Huntington's Disease - news item 1 of 136
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Caudate glucose hypometabolism in a subject carrying an unstable allele of intermediate CAG33 repeat length in the Huntington's disease gene
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(Source: Movement Disorders)
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RSS Source: Med Worm - Huntington's Disease Tue, 1 Mar 2011 11:00:00 pm
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Huntington's Disease - news item 2 of 136
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An item response analysis of the motor and behavioral subscales of the unified Huntington's disease rating scale in Huntington disease gene expansion carriers
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AbstractAlthough the Unified Huntington's Disease Rating Scale (UHDRS) is widely used in the assessment of Huntington disease (HD), the ability of individual items to discriminate individual differences in motor or behavioral manifestations has not been extensively studied in HD gene expansion carriers without a motorâ€defined clinical diagnosis (ie, prodromalâ€HD or...
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RSS Source: Med Worm - Huntington's Disease Tue, 1 Mar 2011 11:00:00 pm
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Huntington's Disease - news item 3 of 136
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THC for Huntington's Disease? CB1 receptors important for more than drug use
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Everyone has heard of some of the medical benefits of marijuana - Glaucoma, anti-nausea, making T.V. funnier - but this early evidence that "weed" might help slow the development of one of our most devestating neurological disorders caught my eye.read more (Source: Psychology Today Addiction Center)
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RSS Source: Med Worm - Huntington's Disease Sat, 26 Feb 2011 1:19:10 am
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Huntington's Disease - news item 4 of 136
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Hope For Patients Following Huntington's Disease Breakthrough
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A huge leap forward in understanding Huntington's disease may give patients hope for a cure. Laboratory tests on skin cells and post-mortem brain tissue of Huntington's disease patients determined that an overactive protein triggers a chain reaction that causes brain nerve cells to die. Toning down the activity of that...
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RSS Source: Med Worm - Huntington's Disease Thu, 24 Feb 2011 8:00:00 am
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Huntington's Disease - news item 5 of 136
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Matter of Life and Death: the Pharmacological Approaches Targeting Apoptosis in Brain Diseases.
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Authors: Cavallucci V, D'Amelio M Neurodegenerative diseases that include amyotrophic lateral sclerosis, Huntington's disease, Alzheimer's disease, Parkinson's disease, stroke, brain trauma and spinal cord injury, are associated with the inappropriate activation of a neuronal cell-suicide program called apoptosis. Given that central nervous system tissue has very limited regenerative capacity it...
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RSS Source: Med Worm - Huntington's Disease Wed, 23 Feb 2011 11:00:00 pm
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Huntington's Disease - news item 6 of 136
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Neuronal degeneration in striatal transplants and Huntington's disease: potential mechanisms and clinical implications
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Cell therapy offers the possibility of replacing degenerated neurons thereby improving the symptoms of neurodegenerative disorders such as Huntington’s disease. However, clinical benefits in patients with Huntington’s disease, if any, have been transient and modest. Grafts survived well at 18 months in one patient with Huntington’s disease, but graft survival...
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RSS Source: Med Worm - Huntington's Disease Wed, 23 Feb 2011 11:00:00 pm
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Huntington's Disease - news item 7 of 136
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Alois Alzheimer (1864-1915) and the Alzheimer syndrome
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Alois Alzheimer is known for his seminal work in recognizing a form of presenile dementia. His early interests were natural history and botany. He started his medical education in Berlin and attended the universities of Wurzburg and Tübingen. Nissl and Alzheimer worked together on extensive investigation of the pathology of...
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RSS Source: Med Worm - Huntington's Disease Wed, 23 Feb 2011 11:00:00 pm
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Huntington's Disease - news item 8 of 136
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Huntington's Disease Breakthrough May Provide Cure
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Huntington's disease breakthrough equals hope for patients - A huge leap forward in understanding Huntington's disease may give patients hope for a cure. (Source: Disabled World)
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RSS Source: Med Worm - Huntington's Disease Wed, 23 Feb 2011 4:55:14 pm
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Huntington's Disease - news item 9 of 136
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Huntington's disease advance: Overactive protein triggers a chain reaction that causes brain nerve cells to die
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A major leap forward in understanding Huntington's disease may give patients hope for a cure. Laboratory tests on skin cells and post-mortem brain tissue of Huntington's disease patients determined that an overactive protein triggers a chain reaction that causes brain nerve cells to die. Toning down the activity of that...
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RSS Source: Med Worm - Huntington's Disease Wed, 23 Feb 2011 9:00:00 am
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Huntington's Disease - news item 10 of 136
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[In Context] Huntington's disease: the crossroads of neurology and psychiatry
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“Hey hey Woody Guthrie I wrote you a songAbout a funny old world that's coming alongSeems sick and it's hungry, it's tired and it's tornIt looks like it's dying and it's hardly been born.†(Source: Lancet Neurology)
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RSS Source: Med Worm - Huntington's Disease Tue, 22 Feb 2011 5:51:33 pm
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Huntington's Disease - news item 11 of 136
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Huntington's disease breakthrough equals hope for patients
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(University of Central Florida) A huge leap forward in understanding Huntington's disease may give patients hope for a cure. (Source: EurekAlert! - Medicine and Health) MedWorm Message: Watch the new MedWorm demo and find out how to get all the very latest, relevant, organized information daily!
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RSS Source: Med Worm - Huntington's Disease Tue, 22 Feb 2011 4:00:00 am
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Huntington's Disease - news item 12 of 136
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Practical Aspects of microRNA Target Prediction.
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Authors: Witkos TM, Koscianska E, Krzyzosia WJ microRNAs (miRNAs) are endogenous non-coding RNAs that control gene expression at the posttranscriptional level. These small regulatory molecules play a key role in the majority of biological processes and their expression is also tightly regulated. Both the deregulation of genes controlled by miRNAs...
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RSS Source: Med Worm - Huntington's Disease Mon, 21 Feb 2011 11:00:00 pm
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Huntington's Disease - news item 13 of 136
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How Disordered Proteins Spread From Cell To Cell, Potentially Spreading Disease
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One bad apple is all it takes to spoil the barrel. And one misfolded protein may be all that's necessary to corrupt other proteins, forming large aggregations linked to several incurable neurodegenerative diseases such as Huntington's, Parkinson's and Alzheimer's. Stanford biology Professor Ron Kopito has shown that the mutant, misfolded...
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RSS Source: Med Worm - Huntington's Disease Mon, 21 Feb 2011 10:00:00 am
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Huntington's Disease - news item 14 of 136
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The physiology, signaling, and pharmacology of dopamine receptors.
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Authors: Beaulieu JM, Gainetdinov RR G protein-coupled dopamine receptors (D1, D2, D3, D4, and D5) mediate all of the physiological functions of the catecholaminergic neurotransmitter dopamine, ranging from voluntary movement and reward to hormonal regulation and hypertension. Pharmacological agents targeting dopaminergic neurotransmission have been clinically used in the management of...
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RSS Source: Med Worm - Huntington's Disease Fri, 18 Feb 2011 10:15:12 am
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Huntington's Disease - news item 15 of 136
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Gene expression profiling of R6/2 transgenic mice with different CAG repeat lengths reveals genes associated with disease onset and progression in Huntington's disease.
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Authors: Tang B, Seredenina T, Coppola G, Kuhn A, Geschwind DH, Luthi-Carter R, Thomas EA R6/2 transgenic mice with expanded CAG repeats (>300) have a surprisingly prolonged disease progression and longer lifespan than prototypical parent R6/2 mice (carrying 150 CAGs), however, the mechanism of this phenotype amelioration is unknown. We...
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RSS Source: Med Worm - Huntington's Disease Wed, 16 Feb 2011 11:00:00 pm
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Huntington's Disease - news item 16 of 136
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Long-term follow-up of pallidal deep brain stimulation in two cases of Huntington's disease
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Conclusion DBS implantation may be a potential treatment option for a subset of HD patients who have significant functional deficits due to chorea. However, appropriate selection of the best candidates for DBS appears to be challenging, given the difficulty in predicting disease course in HD due to its variable nature. (...
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RSS Source: Med Worm - Huntington's Disease Mon, 14 Feb 2011 11:00:00 pm
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Huntington's Disease - news item 17 of 136
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Chemists Make Discovery That May Lead To Drug Treatment Possibilities For Alzheimer's
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UC Santa Barbara scientists have made a discovery that has the potential for use in the early diagnosis and eventual treatment of plaque-related diseases such as Alzheimer's disease and Type 2 diabetes. Their work is published in a recent issue of Nature Chemistry. The amyloid diseases are characterized by plaque...
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RSS Source: Med Worm - Huntington's Disease Mon, 14 Feb 2011 7:00:00 am
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Huntington's Disease - news item 18 of 136
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Embryonic stem cells help deliver 'good genes' in a model of inherited blood disorder
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Researchers report a gene therapy strategy that improves the condition of a mouse model of an inherited blood disorder, beta-thalassemia. Some of the stem cell lines do not inherit the disease gene and can thus be used for transplantation-based treatments of the same mice. The findings could hold promise for...
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RSS Source: Med Worm - Huntington's Disease Sun, 13 Feb 2011 9:00:00 pm
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Huntington's Disease - news item 19 of 136
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Paradoxical function of orexin/hypocretin circuits in a mouse model of Huntington's disease.
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We report that in R6/2 mice, the size of an electrically distinct subpopulation of orexin neurons is reduced, as is the number of orexin-immunopositive cells in some hypothalamic regions. R6/2 orexin cells display altered glutamatergic inputs, and have an abnormal circadian profile of activity, despite normal circadian rhythmicity of the...
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RSS Source: Med Worm - Huntington's Disease Fri, 11 Feb 2011 11:00:00 pm
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Huntington's Disease - news item 20 of 136
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"Brain training" improves cognitive performance and survival in a transgenic mouse model of Huntington's disease.
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"Brain training" improves cognitive performance and survival in a transgenic mouse model of Huntington's disease. Neurobiol Dis. 2011 Feb 12; Authors: Wood NI, Glynn D, Morton AJ Environmental enrichment (EE) has been shown to improve neurological function and cognitive performance in animal models of Alzheimer's disease (AD), Parkinson's disease (PD),...
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RSS Source: Med Worm - Huntington's Disease Fri, 11 Feb 2011 11:00:00 pm
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Huntington's Disease - news item 21 of 136
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New Functions of Ctf18-RFC in Preserving Genome Stability outside Its Role in Sister Chromatid Cohesion
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Author Summary DNA trinucleotide repeats are naturally occurring runs of three base-pairs. Genetic mutations that expand (lengthen) triplet repeats cause multiple neurological diseases, including Huntington's disease. Triplet repeats also contract (shorten) and break. This complex behavior suggests triplet repeats are problematic for DNA replication and repair enzymes. Here, we identified...
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RSS Source: Med Worm - Huntington's Disease Wed, 9 Feb 2011 11:00:00 pm
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Huntington's Disease - news item 22 of 136
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Reflections for February: The Dance
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(Source: Neurology)
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RSS Source: Med Worm - Huntington's Disease Sun, 6 Feb 2011 11:00:00 pm
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Huntington's Disease - news item 23 of 136
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Impaired Long-Term Potentiation in the Prefrontal Cortex of Huntington's Disease Mouse Models: Rescue by D(1) Dopamine Receptor Activation.
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Conclusion: In a previous study, we demonstrated that a deficit in long-term depression (LTD) in the perirhinal cortex could also be reversed by a dopamine agonist. These and our current data indicate that inadequate dopaminergic modulation of cortical synaptic function is an early event in HD and may provide a...
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RSS Source: Med Worm - Huntington's Disease Mon, 31 Jan 2011 11:00:00 pm
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Huntington's Disease - news item 24 of 136
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Scientists To Study RNAi Technology As A Means To Shut Down Production Of A Protein Responsible For Huntington's Disease
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The University of Massachusetts Medical School (UMMS) and Lundbeck Inc.have announced a research collaboration aimed at further development of a targeted therapy to slow or halt the progression of Huntington's disease (HD). At this time, there is no way to stop or reverse the course of HD, a challenging hereditary...
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RSS Source: Med Worm - Huntington's Disease Mon, 31 Jan 2011 8:00:00 am
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Huntington's Disease - news item 25 of 136
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UMMS and Lundbeck to explore potential targeted therapy for Huntington's disease
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(University of Massachusetts Medical School) The University of Massachusetts Medical School and Lundbeck Inc. today announced a research collaboration aimed at further development of a targeted therapy to slow or halt the progression of Huntington's disease. (Source: EurekAlert! - Medicine and Health) MedWorm Message: Watch the new MedWorm demo and...
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RSS Source: Med Worm - Huntington's Disease Fri, 28 Jan 2011 4:00:00 am
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Huntington's Disease - news item 26 of 136
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Longitudinal analysis of the behavioural phenotype in R6/1 (C57BL/6J) Huntington's disease transgenic mice.
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Authors: Brooks SP, Janghra N, Workman VL, Bayram-Weston Z, Jones L, Dunnett SB Huntington's disease is caused by a single mutation on the HTT gene which produces an expansion in the number of glutamine repeats present in the huntingtin protein. This mutation results in an array of motor, cognitive and...
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RSS Source: Med Worm - Huntington's Disease Sun, 23 Jan 2011 11:00:00 pm
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Huntington's Disease - news item 27 of 136
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Proteomic changes in the brains of Huntington's disease mouse models reflect pathology and implicate mitochondrial changes.
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Authors: Deschepper M, Hoogendoorn B, Brooks S, Dunnett SB, Jones L Mouse models of Huntington's disease (HD) have been used extensively to recapitulate the pathological cascade of events in human HD. Mutant huntingtin interacts with many other proteins and has a well documented effect on gene expression. We were interested...
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RSS Source: Med Worm - Huntington's Disease Sun, 23 Jan 2011 11:00:00 pm
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Huntington's Disease - news item 28 of 136
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Immunohistochemical localization of Receptor for Advanced Glycation End products (RAGE) in the R6/2 mouse model of Huntington's disease.
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Authors: Anzilotti S, Giampà C, Laurenti D, Perrone L, Bernardi G, Melone MA, Fusco FR The receptor for advanced glycation end-products (RAGE) is a multi-ligand receptor that belongs to the immunoglobulin superfamily of cell surface receptors, whose ligands are known to be upregulated in neuropathological conditions. RAGE upregulation has been...
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RSS Source: Med Worm - Huntington's Disease Sun, 23 Jan 2011 11:00:00 pm
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Huntington's Disease - news item 29 of 136
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White matter synapses: Form, function, and dysfunction
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Synaptic transmission in the CNS represents the classic mechanism through which neural cells communicate. While vesicular neurotransmitter release has been known to be the preserve of gray matter, it is now known that synaptic-style release of glutamate, the brain's major excitatory neurotransmitter, occurs deep in white matter. Here it permits...
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RSS Source: Med Worm - Huntington's Disease Sun, 23 Jan 2011 11:00:00 pm
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Huntington's Disease - news item 30 of 136
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[Comment] Dutasteride for spinal and bulbar muscular atrophy
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Spinal and bulbar muscular atrophy (SBMA) is a late-onset, hereditary motor neuron disease characterised by slowly progressive muscle weakness and atrophy of the bulbar, facial, and limb muscles. Other neurodegenerative diseases that result from an expanded CAG repeat include Huntington's disease, dentatorubral-pallidoluysian atrophy, and six forms of spinocerebellar ataxia. These...
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RSS Source: Med Worm - Huntington's Disease Thu, 20 Jan 2011 4:28:01 pm
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Huntington's Disease - news item 31 of 136
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New Molecule Could Save Brain Cells From Neurodegeneration, Stroke
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Researchers at the University of North Carolina at Chapel Hill have discovered a molecule that can make brain cells resistant to programmed cell death or apoptosis. This molecule, a tiny strand of nucleotides called microRNA-29 or miR-29, has already been shown to be in short supply in certain neurodegenerative illnesses...
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RSS Source: Med Worm - Huntington's Disease Wed, 19 Jan 2011 9:00:00 am
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Huntington's Disease - news item 32 of 136
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Phenotypic disruption to orofacial movement topography in conditional mutants with generalised CamKIIa/Cre D1Tox vs striatalâ€specific DARPPâ€32/Cre D1Tox ablation of D1 dopamine receptorâ€expressing cells
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AbstractOrofacial movements were quantified in (a) DARPPâ€32/Cre D1Tox mutants, having progressive loss of D1 dopamine receptor expressing striatal medium spiny neurons and (b) CamKIIa/Cre D1Tox mutants, having progressive, generalised loss of forebrain D1 receptor expressing cells. Horizontal jaw movements and tongue protrusions were reduced in DARPPâ€32/Cre but not in CamKIIa/Cre...
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RSS Source: Med Worm - Huntington's Disease Tue, 18 Jan 2011 11:00:00 pm
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Huntington's Disease - news item 33 of 136
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Preclinical study of dimebon on beta-amyloid-mediated neuropathology in Alzheimer's disease
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Conclusion: Collectively, our preclinical studies using the TgCRND8 AD mouse model demonstrated that dimebon might have some beneficial effect in improving cognitive function independent of Alzheimer's disease-type Abeta-related mechanisms or global energy metabolism in the brain. Observations from our study and others suggesting dimebon might improve cognition in wild type...
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RSS Source: Med Worm - Huntington's Disease Tue, 18 Jan 2011 11:00:00 pm
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Huntington's Disease - news item 34 of 136
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New hope in fight against Huntington's Disease
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There is new hope for new ways of treating devastating neurodegenerative disorders such as Huntington's disease. (Source: ScienceDaily Headlines) MedWorm Message: Watch the new MedWorm demo and find out how to get all the very latest, relevant, organized information daily!
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RSS Source: Med Worm - Huntington's Disease Tue, 18 Jan 2011 6:00:00 pm
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Huntington's Disease - news item 35 of 136
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International Stem Cell Applauds Research Showing Parthenogenesis As A Potential Strategy For Treating Inherited Disease
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International Stem Cell Corporation (OTCBB:ISCO), applauds a recent announcement by researchers at Nationwide Children's Hospital showing the use of parthenogenetic stem cells as a potential strategy to treat genetic diseases such as Huntington's disease, beta thalassemia or tuberous sclerosis... (Source: Health News from Medical News Today)
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RSS Source: Med Worm - Huntington's Disease Fri, 14 Jan 2011 12:00:00 pm
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Huntington's Disease - news item 36 of 136
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Novel high-throughput assay to assess cellular manganese levels in a striatal cell line model of Huntington's disease confirms a deficit in manganese accumulation.
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Authors: Kwakye GF, Li D, Bowman AB In spite of the essentiality of manganese (Mn) as a trace element necessary for a variety of physiological processes, Mn in excess accumulates in the brain and has been associated with dysfunction and degeneration of the basal ganglia. Despite the high sensitivity, limited...
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RSS Source: Med Worm - Huntington's Disease Wed, 12 Jan 2011 11:00:00 pm
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Huntington's Disease - news item 37 of 136
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Role of Inositol 1,4,5-Trishosphate Receptors in Pathogenesis of Huntington's Disease and Spinocerebellar Ataxias.
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Authors: Bezprozvanny I Huntington's disease (HD) and spinocerebellar ataxias (SCAs) are autosomal-dominant neurodegenerative disorders. HD is caused by polyglutamine (polyQ) expansion in the amino-terminal region of a protein huntingtin (Htt) and primarily affects medium spiny striatal neurons (MSN). Many SCAs are caused by polyQ-expansion in ataxin proteins and primarily affect...
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RSS Source: Med Worm - Huntington's Disease Wed, 5 Jan 2011 11:00:00 pm
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Huntington's Disease - news item 38 of 136
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Impaired Ideomotor Limb Apraxia in Cortical and Subcortical Dementia: A Comparison of Alzheimer's and Huntington's Disease.
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Conclusions: Ideomotor limb apraxia is a common sign in both groups of patients, occurring in a high percentage. For particular neuropsychological deficits, including ideomotor limb apraxia, a division of dementia in a subcortical and cortical subtype seems to be clinically not meaningful. PMID: 21212634 [PubMed - as supplied by publisher] (...
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RSS Source: Med Worm - Huntington's Disease Tue, 4 Jan 2011 11:00:00 pm
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Huntington's Disease - news item 39 of 136
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Early changes in white matter pathways of the sensorimotor cortex in premanifest huntington's disease
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Conclusions: Disturbances in the white matter connections of the sensorimotor cortex can be demonstrated not only in manifest HD but also in premanifest gene carriers. Connectivity measures are well related to clinical functioning. DTI measures can be regarded as a potential biomarker for HD, due to their ability to objectify...
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RSS Source: Med Worm - Huntington's Disease Sun, 2 Jan 2011 11:00:00 pm
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Huntington's Disease - news item 40 of 136
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Working together: a oneâ€stop shop for Huntington's disease
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AbstractPatients with chronic neurological conditions such as Huntington's disease require the input of a multidisciplinary team to meet their complex needs. Here, the authors describe their specialist clinic in North Staffordshire for patients with Huntington's disease, which offers a oneâ€stop shop for access to health professionals from many different disciplines....
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RSS Source: Med Worm - Huntington's Disease Fri, 31 Dec 2010 11:00:00 pm
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Huntington's Disease - news item 41 of 136
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Longitudinal behavioral, cross-sectional transcriptional and histopathological characterization of a knock-in mouse model of Huntington's disease with 140 CAG repeats.
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Authors: Rising AC, Xu J, Napoli VV, Carlson A, Denovan-Wright EM, Mandel RJ The discovery of the gene mutation responsible for Huntington's disease (HD), huntingtin, in 1993 allowed for a better understanding of the pathology of and enabled the development of animal models. HD is caused by the expansion of...
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RSS Source: Med Worm - Huntington's Disease Mon, 27 Dec 2010 11:00:00 pm
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Huntington's Disease - news item 42 of 136
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Experimental Surgical Therapies for Huntington's Disease.
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Authors: Demeestere J, Vandenberghe W Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder characterized by abnormal movement, cognitive decline, and psychiatric disturbance. HD is caused by a trinucleotide repeat expansion in the HTT gene and a corresponding neurotoxic polyglutamine expansion in the huntingtin protein. There is currently no therapy...
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RSS Source: Med Worm - Huntington's Disease Mon, 27 Dec 2010 11:00:00 pm
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Huntington's Disease - news item 43 of 136
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Early Onset Dementia: Characteristics in a Large Cohort From Academic Memory Clinics
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Conclusions: EOD is characterized by specific features and different causes although Alzheimer's and vascular dementias remain the main causes of dementia in EOD. (C) 2010 Lippincott Williams & Wilkins, Inc. (Source: Alzheimer Disease and Associated Disorders)
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RSS Source: Med Worm - Huntington's Disease Mon, 27 Dec 2010 11:00:00 pm
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Huntington's Disease - news item 44 of 136
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Converging Pathways in the Occurrence of Endoplasmic Reticulum (ER) Stress in Huntington's Disease.
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Authors: Vidal R, Caballero B, Couve A, Hetz C A variety of neurological diseases including Huntington's disease (HD), Alzheimer's disease and Parkinson's disease share common neuropathology, primarily featuring the presence of abnormal protein inclusions containing specific misfolded proteins. Mutations leading to expansion of a poly-glutamine track in Huntingtin cause HD,...
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RSS Source: Med Worm - Huntington's Disease Mon, 27 Dec 2010 11:00:00 pm
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Huntington's Disease - news item 45 of 136
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The neurodegenerative process in a neurotoxic rat model and in patients with Huntington's disease: Histopathological parallels and differences.
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Authors: GunÄová I, Látr I, Mazurová Y Although Huntington's disease (HD) occurs only in humans, the use of animal models is crucial for HD research. New genetic models may provide novel insights into HD pathogenesis, but their relevance to human HD is problematic, particularly owing to a lower number of...
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RSS Source: Med Worm - Huntington's Disease Mon, 27 Dec 2010 11:00:00 pm
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Huntington's Disease - news item 46 of 136
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Role of excitatory amino acid transporterâ€2 (EAAT2) and glutamate in neurodegeneration: Opportunities for developing novel therapeutics
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AbstractGlutamate is an essential excitatory neurotransmitter regulating brain functions. Excitatory amino acid transporter (EAAT)â€2 is one of the major glutamate transporters expressed predominantly in astroglial cells and is responsible for 90% of total glutamate uptake. Glutamate transporters tightly regulate glutamate concentration in the synaptic cleft. Dysfunction of EAAT2 and accumulation...
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RSS Source: Med Worm - Huntington's Disease Mon, 27 Dec 2010 11:00:00 pm
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Huntington's Disease - news item 47 of 136
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Lundbeck Embarks On Research Initiative To Identify And Develop Therapies That May Slow Or Halt The Progression Of Huntington's Disease
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Lundbeck Inc. launched a Huntington's disease (HD) research initiative to identify and ultimately commercialize therapies that may slow or halt the progression of the disease. The research will be driven by collaborations with academic institutions and companies with promising compounds in development. "The Huntington's disease research community has made remarkable...
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RSS Source: Med Worm - Huntington's Disease Mon, 27 Dec 2010 7:00:00 am
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Huntington's Disease - news item 48 of 136
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Smaller intracranial volume in prodromal Huntington's disease: evidence for abnormal neurodevelopment
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Huntington’s disease is an autosomal dominant brain disease. Although conceptualized as a neurodegenerative disease of the striatum, a growing number of studies challenge this classic concept of Huntington’s disease aetiology. Intracranial volume is the tissue and fluid within the calvarium and is a representation of the maximal brain growth obtained...
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RSS Source: Med Worm - Huntington's Disease Thu, 23 Dec 2010 11:00:00 pm
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Huntington's Disease - news item 49 of 136
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Loss of striatal type 1 cannabinoid receptors is a key pathogenic factor in Huntington's disease
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Endocannabinoids act as neuromodulatory and neuroprotective cues by engaging type 1 cannabinoid receptors. These receptors are highly abundant in the basal ganglia and play a pivotal role in the control of motor behaviour. An early downregulation of type 1 cannabinoid receptors has been documented in the basal ganglia of patients...
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RSS Source: Med Worm - Huntington's Disease Thu, 23 Dec 2010 11:00:00 pm
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Huntington's Disease - news item 50 of 136
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Dysfunctions in circadian behavior and physiology in mouse models of Huntington's disease.
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Authors: Kudo T, Schroeder A, Loh DH, Kuljis D, Jordan MC, Roos KP, Colwell CS Many patients with Huntington's disease (HD) exhibit disturbances in their daily cycle of sleep and wake as part of their symptoms. These patients have difficulty sleeping at night and staying awake during the day, which...
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RSS Source: Med Worm - Huntington's Disease Tue, 21 Dec 2010 11:00:00 pm
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Huntington's Disease - news item 51 of 136
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Multi-tract microtransplantation increases the yield of DARPP-32 positive embryonic striatal cells in a rodent model of HuntingtonÂ's disease.
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Authors: Jiang W, Büchele F, Papazoglou A, Döbrössy M, Nikkhah G Embryonic striatal graft mediated functional recovery in the rodent lesion model of Huntington's disease (HD) has been shown to correlate with the proportion of dopamine- and adenosine 3',5'-monophosphat-regulated phosphoprotein with a molecular weight of 32 kDa (DARPP-32) positive neurones...
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RSS Source: Med Worm - Huntington's Disease Tue, 21 Dec 2010 11:00:00 pm
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Huntington's Disease - news item 52 of 136
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Role of MMPâ€9 in striatal bloodâ€brain barrier disruption in a 3â€nitropropionic acid model of Huntington's disease
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Conclusion: Our results highlight the prominent role of MMPâ€9 in BBB disruption in the striatal injured areas of this experimental model of Huntington's disease. (Source: Neuropathology and Applied Neurobiology)
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RSS Source: Med Worm - Huntington's Disease Tue, 21 Dec 2010 11:00:00 pm
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Huntington's Disease - news item 53 of 136
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Differential fate and functional outcome of lithium chloride primed adult neural progenitor cell transplants in a rat model of Huntington's disease
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Conclusions: These initial findings indicate in vitro priming of adult NPCs with lithium chloride may augment transplant efficiency and accelerate sensorimotor function outcome in vivo. (Source: BioMed Central) MedWorm Message: Watch the new MedWorm demo and find out how to get all the very latest, relevant, organized information daily!
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RSS Source: Med Worm - Huntington's Disease Tue, 21 Dec 2010 11:00:00 pm
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Huntington's Disease - news item 54 of 136
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Huntington's disease: a clinical review
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Huntington disease (HD) is a rare neurodegenerative disorder of the central nervous system characterized by unwanted choreatic movements, behavioral and psychiatric disturbances and dementia. Prevalence in the Caucasian population is estimated at 1/10,000-1/20,000. Mean age at onset of symptoms is 30-50 years. In some cases symptoms start before the age...
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RSS Source: Med Worm - Huntington's Disease Sun, 19 Dec 2010 11:00:00 pm
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Huntington's Disease - news item 55 of 136
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In Mouse Model, Transcription Factor Clears Protein Clumps In Huntington's Disease
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Over expressing a transcription factor that promotes the increase in number of mitochondria greatly improves the neurological function of transgenic mice models for Huntington's disease (HD), researchers told the American Society of Cell Biology's 50th Annual Meeting in Philadelphia. Albert La Spada, M.D., Ph.D., and colleagues at the UC San...
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RSS Source: Med Worm - Huntington's Disease Thu, 16 Dec 2010 10:00:00 am
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Huntington's Disease - news item 56 of 136
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In vivo and in vitro imaging of I2 imidazoline receptors in the monkey brain
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AbstractI2 imidazoline receptors (I2Rs) are associated with depression, Alzheimer's disease, and Huntington's disease. However, in vivo imaging of I2Rs in the monkey brain has not been reported until now. We performed in vitro and in vivo imaging of I2Rs in the monkey brain using 11Câ€labeled 2â€(3â€fluoroâ€4â€tolyl)â€4,5â€dihydroâ€1Hâ€imidazole ([11C]FTIMD) which has high...
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RSS Source: Med Worm - Huntington's Disease Tue, 14 Dec 2010 11:00:00 pm
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Huntington's Disease - news item 57 of 136
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[Review] Huntington's disease: from molecular pathogenesis to clinical treatment
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Huntington's disease is a progressive, fatal, neurodegenerative disorder caused by an expanded CAG repeat in the huntingtin gene, which encodes an abnormally long polyglutamine repeat in the huntingtin protein. Huntington's disease has served as a model for the study of other more common neurodegenerative disorders, such as Alzheimer's disease and...
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RSS Source: Med Worm - Huntington's Disease Tue, 14 Dec 2010 6:21:27 am
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Huntington's Disease - news item 58 of 136
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[Articles] Biological and clinical changes in premanifest and early stage Huntington's disease in the TRACK-HD study: the 12-month longitudinal analysis
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Quantitative imaging showed the greatest differentiation across the spectrum of disease and functional measures of decline were sensitive in early HD, with cognitive and quantitative motor impairment also detectable in preHD. We show longitudinal change over 12 months in generalised and regional brain volume, cognition, and quantitative motor tasks in...
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RSS Source: Med Worm - Huntington's Disease Tue, 14 Dec 2010 6:21:26 am
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Huntington's Disease - news item 59 of 136
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[Comment] TRACK and attack Huntington's disease
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Several drugs shown to be neuroprotective in well established experimental models of Parkinson's disease, (HD), and related disorders have not shown relevant disease-modification activity in clinical trials. In some cases, this discrepancy could be related to the differences in the lifespan of human beings with respect to experimental animals and,...
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RSS Source: Med Worm - Huntington's Disease Tue, 14 Dec 2010 6:21:22 am
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Huntington's Disease - news item 60 of 136
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Transcription factor clears protein clumps in Huntington's mice models
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(American Society for Cell Biology) Over expressing a transcription factor that promotes increase in number of mitochondria greatly improves neurological function of transgenic mice models for Huntington's disease. (Source: EurekAlert! - Medicine and Health) MedWorm Message: Watch the new MedWorm demo and find out how to get all the very...
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RSS Source: Med Worm - Huntington's Disease Tue, 14 Dec 2010 4:00:00 am
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Huntington's Disease - news item 61 of 136
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The neurology and natural history of patients with indeterminate CAG repeat length mutations of the Huntington disease gene
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Conclusions: This study documents the disease manifestations and natural history of people with CAG repeat lengths within the indeterminate range. The findings reveal heterogeneity in disease progression and have implications on the advice that should be given to patients and families on risk assessment and prognosis. Long-term follow up of...
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RSS Source: Med Worm - Huntington's Disease Mon, 13 Dec 2010 11:00:00 pm
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Huntington's Disease - news item 62 of 136
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Huntingtin cleavage product A forms in neurons and is reduced by gamma-secretase inhibitors
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Conclusion: We show that cpA and cpB are produced from a larger huntingtin fragment in vivo in mouse brain and in primary neuron cultures. The aspartyl protease involved in forming cpA has cathepsin- D like properties in immortalized neurons and gamma secretase-like properties in primary neurons, suggesting that cell type...
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RSS Source: Med Worm - Huntington's Disease Mon, 13 Dec 2010 11:00:00 pm
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Huntington's Disease - news item 63 of 136
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Endogenous neuroprotection in chronic neurodegenerative disorders: with particular regard to the kynurenines
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AbstractParkinson's disease (PD) and Huntington's disease (HD) are progressive chronic neurodegenerative disorders that are accompanied by a considerable impairment of the motor functions. PD may develop for familial or sporadic reasons, whereas HD is based on a definite genetic mutation. Nevertheless, the pathological processes involve oxidative stress and glutamate excitotoxicity...
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RSS Source: Med Worm - Huntington's Disease Mon, 13 Dec 2010 11:00:00 pm
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Huntington's Disease - news item 64 of 136
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Focal cortical and subcortical atrophy in early Parkinson's disease
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AbstractNeurodegeneration in clinically manifest Parkinson's disease affects the substantia nigra pars compacta, and gradually spreads to the limbic cortices and the neocortex. We used MRI imaging coupled with automated surface reconstruction and segmentation methods to examine cortical thickness and subcortical volumes in nondemented, earlyâ€stage Parkinson's disease patients compared to matched...
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RSS Source: Med Worm - Huntington's Disease Sun, 12 Dec 2010 11:00:00 pm
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Huntington's Disease - news item 65 of 136
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Tapping linked to function and structure in premanifest and symptomatic Huntington disease
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Conclusion: Tapping deficits are evident throughout manifest and premanifest stages. Deficits are more pronounced in later stages and correlate with clinical scores as well as regional brain atrophy, which implies a link between structure and function. The ability to track motor phenotype progression with force-transducer-based tapping measures will be tested...
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RSS Source: Med Worm - Huntington's Disease Sun, 12 Dec 2010 11:00:00 pm
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Huntington's Disease - news item 66 of 136
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Tapping in Huntington disease: A path forward to preventive therapies?
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(Source: Neurology)
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RSS Source: Med Worm - Huntington's Disease Sun, 12 Dec 2010 11:00:00 pm
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Huntington's Disease - news item 67 of 136
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Continuous and Periodic Expansion of CAG Repeats in Huntington's Disease R6/1 Mice
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Author Summary Huntington's disease (HD) is a genetically determined neurodegenerative disorder identified by the presence of a mutation for a long series of CAG repeats (>36 repeats) in the Huntingtin (HTT) gene. Longer repeat sequences cause disease onset at a younger age. The mutation encodes an expanded glutamine tract within...
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RSS Source: Med Worm - Huntington's Disease Wed, 8 Dec 2010 11:00:00 pm
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Huntington's Disease - news item 68 of 136
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Microtiter plate quantification of mutant and wild-type huntingtin normalized to cell count.
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Authors: Weiss A, Grueninger S, Abramowski D, Giorgio FP, Lopatin MM, Rosas HD, Hersch S, Paganetti P Huntington's Disease is caused by a gain-of-function neurotoxic mutation in normally neuroprotective huntingtin. Sensitive assays are required to discriminate mutant from wild-type huntingtin. We developed a normalized 384-plate assay for determination of mutant...
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RSS Source: Med Worm - Huntington's Disease Thu, 2 Dec 2010 11:00:00 pm
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Huntington's Disease - news item 69 of 136
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Impaired Brain Creatine Kinase Activity in Huntington's Disease.
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Conclusion: Our findings lend strong support to the role of impaired energy metabolism in HD, and point out the potential importance of impairment of the CK-catalyzed ATP-buffering system in the etiology of HD. PMID: 21124007 [PubMed - as supplied by publisher] (Source: Neuro-Degenerative Diseases)
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RSS Source: Med Worm - Huntington's Disease Thu, 2 Dec 2010 11:00:00 pm
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Huntington's Disease - news item 70 of 136
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Huntington's Biomarkers Predict Progression Early
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New biomarkers for Huntington's disease may serve as useful targets in future trials evaluating disease-modifying treatments to track progression of patients with premanifest and early Huntington's disease, according to a study published online Dec. 2 in The Lancet Neurology. (Source: Modern Medicine) MedWorm Message: Watch the new MedWorm demo and...
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RSS Source: Med Worm - Huntington's Disease Thu, 2 Dec 2010 11:00:00 pm
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Huntington's Disease - news item 71 of 136
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Study Identifies Potential Huntington's Disease Biomarkers
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Early biomarkers of Huntington's disease identified in patients long before disease symptoms become apparent. Medscape Medical News (Source: Medscape Today Headlines)
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RSS Source: Med Worm - Huntington's Disease Thu, 2 Dec 2010 3:36:41 pm
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Huntington's Disease - news item 72 of 136
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MRI Markers Track Huntington's Progression (CME/CE)
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(MedPage Today) -- Changes in brain volume and performance on cognition and motor function tests occur early in patients with Huntington's disease and can be quantified over a one-year period, researchers said. (Source: MedPage Today Neurology)
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RSS Source: Med Worm - Huntington's Disease Thu, 2 Dec 2010 2:53:59 pm
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Huntington's Disease - news item 73 of 136
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Huntington's symptoms appear a decade before diagnosis
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Simple drawing tasks can be used to help identify people with Huntington's disease around 10 years before clinical diagnosis (Source: New Scientist - Health) MedWorm Message: Watch the new MedWorm demo and find out how to get all the very latest, relevant, organized information daily!
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RSS Source: Med Worm - Huntington's Disease Thu, 2 Dec 2010 1:38:21 pm
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Huntington's Disease - news item 74 of 136
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Study Identifies Promising Biomarkers For Huntington's Disease That Could Be Used To Test Disease-Modifying Treatments (Track-HD Study)
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A range of new clinical, functional, and neuroimaging tests make it possible to track the progression of Huntington's disease (HD) long before noticeable symptoms appear, providing useful biomarkers (indicators) that could be used in future trials to detect the effectiveness of potential disease-modifying treatments within a short time period. These...
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RSS Source: Med Worm - Huntington's Disease Thu, 2 Dec 2010 8:00:00 am
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Huntington's Disease - news item 75 of 136
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Study says Huntington's tests to aid drug search
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LONDON (Reuters) - Scientists have developed a new range of tests that allow them to track the progression of Huntington's disease long before symptoms appear and should aid the development of drugs for the incurable inherited disease. (Source: Reuters: Health)
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RSS Source: Med Worm - Huntington's Disease Wed, 1 Dec 2010 11:09:30 pm
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Huntington's Disease - news item 76 of 136
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In this issue: Biotechnology Journal 12/2010.
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Authors: HUMAN DISEASES IN C. ELEGANS: Markaki and Tavernarakis, et al., Biotechnol. J. 2010, 5, 1261-1276The worm Caenorhabditis elegans is one of the most versatile and powerful model organisms. Already in the early 1960's it was introduced by Sydney Brenner to study development and neurobiology. It is a free-living, non-parasitic...
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RSS Source: Med Worm - Huntington's Disease Tue, 30 Nov 2010 11:00:00 pm
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Huntington's Disease - news item 77 of 136
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Could successful (mitochondrial) networking help prevent Huntington's disease?
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AbstractPolyglutamine expansions in huntingtin (Htt) are known to cause the profound neurodegenerative disorder, Huntington's disease (HD). Mitochondrial dysfunction has long been implicated in the pathophysiology of HD, but the underlying mechanism remains obscure. An article by Costa et al in this months edition describes a smooth mechanistic cascade from the...
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RSS Source: Med Worm - Huntington's Disease Sun, 28 Nov 2010 11:00:00 pm
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Huntington's Disease - news item 78 of 136
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Novel Method Results In Promising Drugs For Huntington's Disease Therapy
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Huntington's disease (HD) is an incurable progressive neurodegenerative genetic disorder which affects motor coordination and leads to cognitive decline and dementia. The disease pathology stems from a mutation in the huntingtin (Htt) gene which results in the accumulation of toxic proteins leading to neuronal cell death. Earlier studies have clearly...
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RSS Source: Med Worm - Huntington's Disease Thu, 25 Nov 2010 9:00:00 am
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Huntington's Disease - news item 79 of 136
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Altered emotional and motivational processing in the transgenic rat model for Huntington's disease.
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Authors: Faure A, Höhn S, Von Hörsten S, Delatour B, Raber K, Le Blanc P, Desvignes N, Doyère V, El Massioui N Huntington disease (HD) is caused by an expansion of CAG repeat in the Huntingtin gene. Patients demonstrate a triad of motor, cognitive and psychiatric symptoms. A transgenic rat...
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RSS Source: Med Worm - Huntington's Disease Tue, 23 Nov 2010 11:00:00 pm
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Huntington's Disease - news item 80 of 136
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Allele-Selective Inhibition of Huntingtin Expression by Switching to an miRNA-like RNAi Mechanism.
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Authors: Hu J, Liu J, Corey DR Inhibiting expression of huntingtin (HTT) protein is a promising strategy for treating Huntington's disease (HD), but indiscriminant inhibition of both wild-type and mutant alleles may lead to toxicity. An ideal silencing agent would block expression of mutant HTT while leaving expression of wild-type HTT...
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RSS Source: Med Worm - Huntington's Disease Tue, 23 Nov 2010 11:00:00 pm
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Huntington's Disease - news item 81 of 136
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Identification and Evaluation of Small Molecule Pan-Caspase Inhibitors in Huntington's Disease Models.
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In this study, the identified nonpeptidic caspase inhibitors were used to confirm the role of caspase-mediated Htt proteolysis in HD. These results further implicate caspases as promising targets for HD therapeutic development. PMID: 21095569 [PubMed - in process] (Source: Chemistry and Biology)
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RSS Source: Med Worm - Huntington's Disease Tue, 23 Nov 2010 11:00:00 pm
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Huntington's Disease - news item 82 of 136
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The genetics of epilepsy†The past, the present and future
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Abstract: A brief history of human genetics: Sixty years is an appropriate yardstick for many reasons, not least for the remarkable advances in medicine, public health, psychology and biological disciplines. Particularly relevant is the approaching 60th anniversary of the discovery of the structure of DNA, which unlocked the driving force...
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RSS Source: Med Worm - Huntington's Disease Sun, 21 Nov 2010 11:00:00 pm
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Huntington's Disease - news item 83 of 136
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Dendritic spine loss and neurodegeneration is rescued by Rab11 in models of Huntington's disease
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Authors: P Richards, C Didszun, S Campesan, A Simpson, B Horley, K W Young, P Glynn, K Cain, C P Kyriacou, F Giorgini & P Nicotera (Source: Cell Death and Differentiation)
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RSS Source: Med Worm - Huntington's Disease Thu, 18 Nov 2010 11:00:00 pm
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Huntington's Disease - news item 84 of 136
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[Correspondence] Accurate prevalence and uptake of testing for Huntington's disease
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Laura Spinney comments on the apparent lack of accurate figures on the prevalence and number of people at risk of Huntington's disease in the UK. Most studies were done more than 20 years ago, and the most recent study was published by myself and my colleagues in 1995. This latest...
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RSS Source: Med Worm - Huntington's Disease Wed, 17 Nov 2010 9:50:17 am
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Huntington's Disease - news item 85 of 136
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Natural Compound Shows Promise Against Huntington's Disease
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Fisetin, a naturally occurring compound found in strawberries and other fruits and vegetables, slows the onset of motor problems and delays death in three models of Huntington's disease, according to researchers at the Salk Institute for Biological Studies. The study, published in the online edition of Human Molecular Genetics, sets...
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RSS Source: Med Worm - Huntington's Disease Tue, 16 Nov 2010 8:00:00 am
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Huntington's Disease - news item 86 of 136
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Up-regulation of the isoenzymes MAO-A and MAO-B in the human basal ganglia and pons in Huntington's disease revealed by quantitative enzyme radioautography.
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Authors: Richards G, Messer J, Waldvogel HJ, Gibbons HM, Dragunow M, Faull RL, Saura J Huntington's disease (HD) is a rare genetic disease associated with the degeneration of GABAergic striatal projection neurons in the basal ganglia leading to movement disorders with behavioural symptoms for which there is presently no therapy....
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RSS Source: Med Worm - Huntington's Disease Wed, 10 Nov 2010 11:00:00 pm
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Huntington's Disease - news item 87 of 136
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A myriad of miRNA variants in control and Huntington's disease brain regions detected by massively parallel sequencing
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Huntington disease (HD) is a neurodegenerative disorder that predominantly affects neurons of the forebrain. We have applied the Illumina massively parallel sequencing to deeply analyze the small RNA populations of two different forebrain areas, the frontal cortex (FC) and the striatum (ST) of healthy individuals and individuals with HD. More...
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RSS Source: Med Worm - Huntington's Disease Tue, 9 Nov 2010 11:00:00 pm
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Huntington's Disease - news item 88 of 136
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The number of CAG repeats within the normal allele does not influence the age of onset in Huntington's disease
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AbstractHuntington's disease (HD) is caused by the expansion of the number of CAG repeats on the chromosome 4p16.3, which results in elongated glutamine tract of huntingtin. The purpose of this work was to examine the interaction between the normal and mutant alleles of this gene and their effect on the...
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RSS Source: Med Worm - Huntington's Disease Tue, 9 Nov 2010 11:00:00 pm
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Huntington's Disease - news item 89 of 136
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Reflections for November: Love in the time of Huntington's disease
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(No abstract is available for this citation) (Source: Neurology)
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RSS Source: Med Worm - Huntington's Disease Sun, 7 Nov 2010 11:00:00 pm
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Huntington's Disease - news item 90 of 136
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Magnetic resonance spectroscopy biomarkers in premanifest and early Huntington disease
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Conclusions: We demonstrate lower putaminal tNAA in early HD compared to controls in a cross-section of subjects. A novel biomarker role for mI in early HD was also identified. These findings resolve disagreement in the literature about the role of MRS as an HD biomarker. We conclude that putaminal MRS...
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RSS Source: Med Worm - Huntington's Disease Sun, 7 Nov 2010 11:00:00 pm
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Huntington's Disease - news item 91 of 136
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A(2A) receptor knockout worsens survival and motor behaviour in a transgenic mouse model of Huntington's disease.
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Authors: Mievis S, Blum D, Ledent C Huntington's disease (HD) is a progressive neurodegenerative genetic disorder which leads to motor, cognitive and psychiatric disturbances. The primary neuropathological hallmark is atrophy of the striatum. HD preferentially affects efferent striato-pallidal neurons that express enkephalin as well as dopamine D2 and A(2A) adenosine...
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RSS Source: Med Worm - Huntington's Disease Fri, 5 Nov 2010 11:00:00 pm
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Huntington's Disease - news item 92 of 136
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The Common Inhaled Anesthetic Isoflurane Increases Aggregation of Huntingtin and Alters Calcium Homeostasis in a Cell Model of Huntington's Disease.
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Authors: Wang Q, Liang G, Yang H, Wang S, Eckenhoff MF, Wei H Isoflurane is known to increase β-amyloid aggregation and neuronal damage. We hypothesized that isoflurane will have similar effects on the polyglutamine huntingtin protein, and will cause alterations in intracellular calcium homeostasis. We tested this hypothesis in striatal...
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RSS Source: Med Worm - Huntington's Disease Thu, 4 Nov 2010 11:00:00 pm
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Huntington's Disease - news item 93 of 136
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Proximal Movements Compensate for Distal Forelimb Movement Impairments in a Reach-To-Eat Task in Huntington's Disease: New Insights into Motor Impairments in a Real-World Skill.
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Authors: Klein A, Sacrey LA, Dunnett SB, Whishaw IQ, Nikkhah G Huntington's disease (HD) causes severe motor impairments that are characterized by chorea, dystonia, and impaired fine motor control. The motor deficits include deficits in the control of the forelimb, but as yet there has been no comprehensive assessment of...
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RSS Source: Med Worm - Huntington's Disease Thu, 4 Nov 2010 11:00:00 pm
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Huntington's Disease - news item 94 of 136
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Huntington's Disease: from Molecular Basis to Therapeutic Advances.
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Authors: Krobitsch S, Kazantsev AG Huntington's disease is an autosomal dominant genetic neurodegenerative disorder, which is characterized by progressive motor dysfunction, emotional disturbances, dementia, and weight loss. The disease is caused by pathological CAG-triplet repeat extension(s), encoding polyglutamines, within the gene product, huntingtin. Huntingtin is ubiquitously expressed through the body...
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RSS Source: Med Worm - Huntington's Disease Tue, 2 Nov 2010 11:00:00 pm
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Huntington's Disease - news item 95 of 136
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Does reduced [123I]-FP-CIT binding in Huntington's disease suggest pre-synaptic dopaminergic involvement?
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Conclusion: This is the first study to use in vivo [123I]-FP-CIT/SPECT imaging to confirm prior descriptions using PET of a pre-synaptic dopaminergic system defect in HD. (Source: Clinical Neurology and Neurosurgery)
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RSS Source: Med Worm - Huntington's Disease Tue, 2 Nov 2010 1:18:44 am
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Huntington's Disease - news item 96 of 136
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Lack of interleukin-1 type 1 receptor enhances the accumulation of mutant huntingtin in the striatum and exacerbates the neurological phenotypes of Huntington's disease mice
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Huntington's disease results from expansion of a glutamine repeat (>36 glutamines) in the N-terminal region of huntingtin (htt) and is characterized by preferential neurodegeneration in the striatum of the brain. N171-82Q mice that express N-terminal 171 amino acids of htt with an 82-glutamine repeat show severe neurological phenotypes and die...
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RSS Source: Med Worm - Huntington's Disease Mon, 1 Nov 2010 11:00:00 pm
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Huntington's Disease - news item 97 of 136
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Functional Increase of Brain Histaminergic Signaling in Huntington's Disease
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AbstractTo evaluate whether central histaminergic signaling in Huntington's disease (HD) patients is affected, which may contribute to a number of debilitating signs and symptoms, we assessed the mRNA levels of histidine decarboxylase (HDC) and the volume and neuron number in the hypothalamic tuberomamillary nucleus (TMN) (HD n = 8, controls n =...
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RSS Source: Med Worm - Huntington's Disease Sun, 31 Oct 2010 11:00:00 pm
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Huntington's Disease - news item 98 of 136
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Interview.
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Authors: Barker R Dr Roger A Barker is a University Reader in Clinical Neuroscience and Honorary Consultant in Neurology at the Addenbrooke's Hospital. He trained at Oxford and in London and has been in his current position for 10 years, having completed an MRC Clinician Scientist Fellowship prior to this....
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RSS Source: Med Worm - Huntington's Disease Sun, 31 Oct 2010 11:00:00 pm
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Huntington's Disease - news item 99 of 136
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Mesenchymal stem cells for the treatment of neurodegenerative disease.
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Authors: Joyce N, Annett G, Wirthlin L, Olson S, Bauer G, Nolta JA Mesenchymal stem cells/marrow stromal cells (MSCs) present a promising tool for cell therapy, and are currently being tested in US FDA-approved clinical trials for myocardial infarction, stroke, meniscus injury, limb ischemia, graft-versus-host disease and autoimmune disorders. They...
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RSS Source: Med Worm - Huntington's Disease Sun, 31 Oct 2010 11:00:00 pm
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Huntington's Disease - news item 100 of 136
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Mitochondrial fission and cristae disruption increase the response of cell models of Huntington's disease to apoptotic stimuli
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In conclusion, the cristae remodelling of the fragmented HD mitochondria contributes to their hypersensitivity to apoptosis.See accompanying Closeup by Oliveira and Lightowlers DOI 10.1002/emmm.201000104. (Source: EMBO Molecular Medicine)
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RSS Source: Med Worm - Huntington's Disease Sun, 31 Oct 2010 11:00:00 pm
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Huntington's Disease - news item 101 of 136
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Huntington's Disease is a Disorder of the Corpus Striatum: Focus on Rhes.
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Authors: Subramaniam S, Snyder SH Despite identification of the gene for huntingtin (Htt) as causal in Huntington's Disease (HD), explication of HD symptoms and selective damage to the corpus striatum has been elusive. The small G protein Rhes, highly localized to the striatum binds selectively to mutant Htt (mHtt) and...
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RSS Source: Med Worm - Huntington's Disease Fri, 29 Oct 2010 11:00:00 pm
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Huntington's Disease - news item 102 of 136
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[Correspondence] Huntington's disease
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Michael Rawlins (Oct 23, p 1372) comments that the prevalence of Huntington's disease is rising. We would like to suggest several additional explanations for this finding.First, the baby-boomers are now in their 50s†60s. Typically Huntington's disease begins in the 30s†40s, but given the duration of illness, peak prevalence...
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RSS Source: Med Worm - Huntington's Disease Fri, 29 Oct 2010 6:07:15 pm
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Huntington's Disease - news item 103 of 136
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Huntington's Disease and Behavioral Dyscontrol [LETTERS TO THE EDITOR]
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(No abstract is available for this citation) (Source: J Neuropsychiatry Clin Neurosci) MedWorm Message: Watch the new MedWorm demo and find out how to get all the very latest, relevant, organized information daily!
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RSS Source: Med Worm - Huntington's Disease Thu, 28 Oct 2010 11:00:00 pm
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Huntington's Disease - news item 104 of 136
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Delayed Huntington's Disease Diagnosis in Two Alcoholic Patients With a Family History of "Parkinson's Disease" [LETTERS TO THE EDITOR]
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(No abstract is available for this citation) (Source: J Neuropsychiatry Clin Neurosci)
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RSS Source: Med Worm - Huntington's Disease Thu, 28 Oct 2010 11:00:00 pm
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Huntington's Disease - news item 105 of 136
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Altered microRNA regulation in Huntington's disease models.
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Authors: Lee ST, Chu K, Im WS, Yoon HJ, Im JY, Park JE, Park KH, Jung KH, Lee SK, Kim M, Roh JK Huntington's disease (HD) is a genetic neurodegenerative disease caused by abnormal CAG expansion. microRNAs (miRNAs) are short RNA molecules regulating gene expression, and are implicated in a...
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RSS Source: Med Worm - Huntington's Disease Tue, 26 Oct 2010 11:00:00 pm
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Huntington's Disease - news item 106 of 136
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Fall risk assessment using the Tinetti mobility test in individuals with Huntington's disease. - Kloos AD, Kegelmeyer DA, Young GS, Kostyk SK.
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This study examined the concurrent validity, usefulness of the TMT as a fall risk screening tool, and the potential ability of the TMT to predict fa... (Source: SafetyLit: All (Unduplicated))
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RSS Source: Med Worm - Huntington's Disease Sun, 24 Oct 2010 8:20:09 am
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Huntington's Disease - news item 107 of 136
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Brief Ampakine Treatments Slow the Progression of Huntington's Disease Phenotypes in R6/2 Mice.
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Authors: Simmons DA, Mehta RA, Lauterborn JC, Gall CM, Lynch G Daily, systemic injections of a positive AMPA-type glutamate receptor modulator (ampakine) have been shown to reduce synaptic plasticity defects in rodent models of aging and early-stage Huntington's Disease (HD). Here we report that long-term ampakine treatment markedly slows the...
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RSS Source: Med Worm - Huntington's Disease Fri, 22 Oct 2010 10:00:00 pm
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Huntington's Disease - news item 108 of 136
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[Comment] Huntington's disease out of the closet?
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In her Art of Medicine essay in The Lancet today, Alice Wexler recounts the stigma faced by those with Huntington's disease, a cruelly progressive and incurable neurodegenerative condition that usually (not invariably) presents in mid-life. The disease is inherited as an autosomal dominant trait so that children of a parent...
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RSS Source: Med Worm - Huntington's Disease Fri, 22 Oct 2010 10:00:00 pm
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Huntington's Disease - news item 109 of 136
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Systemic energy homeostasis in Huntington's disease patients
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Conclusion These findings suggest sympathetic hyperactivity as an underlying mechanism of increased energy expenditure in HD, as well as peripheral polyglutamine length dependent interference of mutant huntingtin with insulin signalling that may become clinically relevant in carriers of mutations with large CAG repeat sizes. (Source: Journal of Neurology, Neurosurgery and...
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RSS Source: Med Worm - Huntington's Disease Thu, 21 Oct 2010 10:00:00 pm
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Huntington's Disease - news item 110 of 136
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POMD07 Quantitative assessment of biological and clinical manifestations of Huntington's disease before and after diagnosis--the TRACK-HD study
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Conclusions Our results provide evidence for quantifiable biological and clinical alterations in HD expansion carriers compared to age-matched controls. Many parameters differ from age-matched controls in a graded fashion showing changes of increasing magnitude covering two decades of disease development from pre-HD up to ~16 years from predicted onset through...
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RSS Source: Med Worm - Huntington's Disease Thu, 21 Oct 2010 10:00:00 pm
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Huntington's Disease - news item 111 of 136
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PAW36 Oculomotor deficits in presymptomatic and early Huntington's disease and their structural brain correlates
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Conclusions Together these results show the validity of oculomotor testing in presymptomatic HD and the functional consequences of widespread neurodegeneration associated with HD. (Source: Journal of Neurology, Neurosurgery and Psychiatry) MedWorm Message: Watch the new MedWorm demo and find out how to get all the very latest, relevant, organized information...
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RSS Source: Med Worm - Huntington's Disease Thu, 21 Oct 2010 10:00:00 pm
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Huntington's Disease - news item 112 of 136
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UCI stem cell researchers to receive $9.35 million in state funding
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(University of California -- Irvine) UC Irvine scientists will receive grants totaling $9.35 million to help create stem cell treatments for retinitis pigmentosa, Huntington's disease and traumatic brain injury. (Source: EurekAlert! - Medicine and Health)
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RSS Source: Med Worm - Huntington's Disease Thu, 21 Oct 2010 3:00:00 am
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Huntington's Disease - news item 113 of 136
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Targeting Kynurenine 3-Monooxygenase (KMO): Implications for Therapy in Huntington's Disease.
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Authors: Thevandavakkam MA, Schwarcz R, Muchowski PJ, Giorgini F Huntington's disease (HD) is an adult onset neurodegenerative disease caused by a polyglutamine expansion in the huntingtin protein Recent work has shown that perturbation of kynurenine pathway (KP) metabolism is a hallmark of HD pathology, and that changes in brain levels...
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RSS Source: Med Worm - Huntington's Disease Wed, 20 Oct 2010 2:40:43 am
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Huntington's Disease - news item 114 of 136
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Autophagy in the Central Nervous System.
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Authors: Xilouri M, Stefanis L The autophagy-lysosomal pathway is a major proteolytic pathway that in mammalian systems mainly comprises of macroautophagy and chaperone-mediated autophagy. The former is relatively non-selective and involves bulk degradation of proteins and organelles, whereas the latter is selective for certain cytosolic proteins. These autophagy pathways are...
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RSS Source: Med Worm - Huntington's Disease Wed, 20 Oct 2010 2:40:19 am
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Huntington's Disease - news item 115 of 136
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External globus pallidus stimulation modulates brain connectivity in Huntington's disease
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Authors: Noémie Ligot, Pierre Krystkowiak, Clémence Simonin, Serge Goldman, Philippe Peigneux, John Van Naemen, Michel Monclus, Simon Frédéric Lacroix, David Devos, Kathy Dujardin, Christine Delmaire, Eric Bardinet, Arnaud Delval, Marie Delliaux, Luc Defebvre, Jerome Yelnik, Serge Blond, Alain Destée & Xavier De Tiège (Source: Journal of Cerebral Blood Flow)
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RSS Source: Med Worm - Huntington's Disease Tue, 19 Oct 2010 10:00:00 pm
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Huntington's Disease - news item 116 of 136
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Glaxo to Invest More in Drugs for Rare Diseases
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From Associated Press (October 18, 2010) LONDON -- GlaxoSmithKline says it will devote more resources to developing new drugs for rare illnesses including Huntington's disease, Duchenne muscular dystrophy, and hard-to-treat cancers. The... (Source: Drugs.com - Pharma News)
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RSS Source: Med Worm - Huntington's Disease Mon, 18 Oct 2010 2:15:45 pm
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Huntington's Disease - news item 117 of 136
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Kathleen Edward: Dying Girl Cyberbullied
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Community Rallies for 7-Year-Old Fighting Huntington's Disease and Vicious Taunts (Source: Health News: CBSNews.com)
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RSS Source: Med Worm - Huntington's Disease Wed, 13 Oct 2010 7:19:24 pm
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Huntington's Disease - news item 118 of 136
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An intrastriatal brain-derived neurotrophic factor infusion restores striatal gene expression in Bdnf heterozygous mice.
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In this study, the possibility that intrastriatal infusions of BDNF would elevate gene expression in the striatum of Bdnf (+/-) mice was investigated. Wildtype and Bdnf (+/-) mice received a single, bilateral microinjection of BDNF or PBS into the dorsal striatum. Mice were killed 24Â h later and semi-quantitative in situ...
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RSS Source: Med Worm - Huntington's Disease Mon, 11 Oct 2010 10:00:00 pm
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Huntington's Disease - news item 119 of 136
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Low anaerobic threshold and increased skeletal muscle lactate production in subjects with Huntington's disease
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Abstract (Source: Movement Disorders)
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RSS Source: Med Worm - Huntington's Disease Fri, 8 Oct 2010 3:41:52 pm
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Huntington's Disease - news item 120 of 136
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Gaining and maintaining consent when capacity can be an issue: a research study with people with Huntington's disease
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In conclusion, this paper adds a pragmatic approach to the debate on informed consent by describing the development of a written information sheet and consent form being used in a current social research study. Particular emphasis is placed on the importance of written information being adapted according to the needs...
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RSS Source: Med Worm - Huntington's Disease Mon, 4 Oct 2010 9:20:01 pm
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Huntington's Disease - news item 121 of 136
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British IVF pioneer, Robert Edwards, wins Nobel prize for medicine
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Robert Edwards, the British scientist who pioneered IVF, was responsible for the conception of Louise Brown, the world's first test-tube babyThe Nobel prize for physiology or medicine for 2010 has been awarded to the British scientist who pioneered in-vitro fertilisation, a procedure that has helped in the conception and birth...
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RSS Source: Med Worm - Huntington's Disease Mon, 4 Oct 2010 10:23:13 am
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Huntington's Disease - news item 122 of 136
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Neurocognitive signs in prodromal Huntington disease.
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Conclusions: Neurocognitive tests are robust clinical indicators of the disease process prior to reaching criteria for motor diagnosis of HD. (PsycINFO Database Record (c) 2010 APA, all rights reserved) (Source: Neuropsychology)
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RSS Source: Med Worm - Huntington's Disease Sun, 3 Oct 2010 11:00:00 pm
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Huntington's Disease - news item 123 of 136
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Mitochondrial-associated metabolic changes and neurodegeneration in Huntington's disease - from clinical features to the bench.
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Authors: Rosenstock TR, Duarte AI, Rego AC Huntington's disease (HD) is a genetic neurodegenerative disease selectively leading to striatal neurodegeneration, but also affecting the cortex and the hypothalamus. Although it is hard to predict the sequence of cell-damaging events occurring in HD patients, several pathological mechanisms have been proposed to...
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RSS Source: Med Worm - Huntington's Disease Thu, 30 Sep 2010 5:11:00 pm
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Huntington's Disease - news item 124 of 136
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The role of PGC-1α in the pathogenesis of neurodegenerative disorders.
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Authors: Róna-Vörös K, Weydt P Mitochondrial dysfunction is a common hallmark of ageing-related diseases involving neurodegeneration. Huntington's disease (HD) is one of the most common monogenetic forms of neurodegenerative disorders and shares many salient features with the major sporadic disease of neurodegeneration, such as amyotrophic lateral sclerosis (ALS), Alzheimer's disease (...
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RSS Source: Med Worm - Huntington's Disease Thu, 30 Sep 2010 5:10:51 pm
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Huntington's Disease - news item 125 of 136
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Sirtuins: common targets in aging and in neurodegeneration.
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Authors: de Oliveira RM, Pais TF, Outeiro TF Aging has been a subject of interest since primordial times. More recently, it became clear that aging is the major known risk factor for several neurodegenerative disorders, such as Alzheimer's disease, Parkinson's disease and Huntington's disease. A major focus in the field...
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RSS Source: Med Worm - Huntington's Disease Thu, 30 Sep 2010 5:10:48 pm
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Huntington's Disease - news item 126 of 136
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Progress and prospects: stem cells and neurological diseases
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Authors: S Gögel, M Gubernator & S L Minger (Source: Gene Therapy)
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RSS Source: Med Worm - Huntington's Disease Wed, 29 Sep 2010 10:00:00 pm
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Huntington's Disease - news item 127 of 136
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The Role of Natural Products in the Discovery of New Drug Candidates for the Treatment of Neurodegenerative Disorders I: Parkinson's Disease.
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Authors: Campos HC, da Rocha MD, Viegas FP, Nicastro PC, Fossaluzza PC, Fraga CA, Barreiro EJ, Viegas C Neurodegenerative disorders such as Parkinson's disease (PD), Alzheimer's disease (AD), Huntington's disease (HD) and amyotrophic lateral sclerosis (ALS) are currently incurable pathologies with huge social and economic impacts closely related to the...
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RSS Source: Med Worm - Huntington's Disease Tue, 28 Sep 2010 10:00:00 pm
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Huntington's Disease - news item 128 of 136
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Evidence for a predisposing background for CAG expansion leading to HTT mutation in a Chinese population
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Conclusion: Our findings indicate that HTT mutation is likely of multiple origins in the Chinese population. Among the origins, more new HTT mutations may arise from the (CCG)10 than from other CCG alleles, which suggests that the (CCG)10 allele may represent a predisposing background for CAG expansion in Chinese populations....
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RSS Source: Med Worm - Huntington's Disease Mon, 27 Sep 2010 10:00:00 pm
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Huntington's Disease - news item 129 of 136
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Getting Off Tract: Polyglutamine Disease Involves Other Regions Of Protein
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Many genes code for proteins that have a "polyglutamine tract," several glutamine amino acid residues in a row. Nine inherited neurodegenerative diseases, including Huntington's disease and spinocerebellar ataxia type 1 (SCA1), are associated with mutations that cause abnormally long polyglutamine tracts. One theory suggests that accumulation of proteins with extra...
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RSS Source: Med Worm - Huntington's Disease Fri, 24 Sep 2010 8:00:00 am
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Huntington's Disease - news item 130 of 136
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Huntington's insight given by stem cells
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Studying Huntington's disease in human embryonic stem cells show signs of the disease in cells just a few days old, Australian researchers say. (Source: CBC | Health)
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RSS Source: Med Worm - Huntington's Disease Wed, 22 Sep 2010 3:14:31 pm
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Huntington's Disease - news item 131 of 136
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To scientists, he's the real rock star
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Dr. Francis Collins has discovered the genes that cause cystic fibrosis and Huntington's disease and runs the National Institutes of Health. But hand him a guitar and he becomes Dr. Rock 'n' Roll.Considering that he's the director of the federal agency that invests more than $30 billion in medical research...
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RSS Source: Med Worm - Huntington's Disease Sat, 18 Sep 2010 6:00:00 am
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Huntington's Disease - news item 132 of 136
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Technical factors that influence neural transplant safety in Huntington's disease.
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Authors: Freeman TB, Cicchetti F, Bachoud-Lévi AC, Dunnett SB PMID: 20849848 [PubMed - as supplied by publisher] (Source: Experimental Neurology) MedWorm Message: Watch the new MedWorm demo and find out how to get all the very latest, relevant, organized information daily!
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RSS Source: Med Worm - Huntington's Disease Wed, 15 Sep 2010 10:00:00 pm
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Huntington's Disease - news item 133 of 136
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Early synaptic pathophysiology in neurodegeneration: insights from Huntington's disease.
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Authors: Milnerwood AJ, Raymond LA Investigations of synaptic transmission and plasticity in mouse models of Huntington's disease (HD) demonstrate neuronal dysfunction long before the onset of classical disease indicators. Similarly, recent human studies reveal synaptic dysfunction decades before predicted clinical diagnosis in HD gene carriers. These studies guide premanifest tracking...
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RSS Source: Med Worm - Huntington's Disease Tue, 14 Sep 2010 10:00:00 pm
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Huntington's Disease - news item 134 of 136
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Neurobiology: Neuronal housekeeping
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Nature 467, 255 (2010). doi:10.1038/467255c Proc. Natl Acad. Sci. USA doi:10.1073/pnas.1004498107 (2010)Neurodegenerative disorders such as Huntington's disease are characterized by the accumulation of misfolded, defective proteins in brain cells. Certain drugs can stimulate a process for clearing such proteins, called autophagy, in some cells, (Source: Nature)
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RSS Source: Med Worm - Huntington's Disease Tue, 14 Sep 2010 10:00:00 pm
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Huntington's Disease - news item 135 of 136
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Reply to Valenza and Cattaneo: SIRT2-mediated neuroprotection and cholesterol dyshomeostasis in Huntington's disease [Letters]
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(No abstract is available for this citation) (Source: Proceedings of the National Academy of Sciences)
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RSS Source: Med Worm - Huntington's Disease Tue, 14 Sep 2010 3:44:41 pm
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Huntington's Disease - news item 136 of 136
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Neuroprotection and brain cholesterol biosynthesis in Huntington's disease [Letters]
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(No abstract is available for this citation) (Source: Proceedings of the National Academy of Sciences)
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RSS Source: Med Worm - Huntington's Disease Tue, 14 Sep 2010 3:44:41 pm
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