Mutant huntingtin can paradoxically protect neurons from death
نویسندگان
چکیده
منابع مشابه
Selective inhibitors of death in mutant huntingtin cells.
Huntington disease (HD) is an inherited neurodegenerative disorder with unclear pathophysiology. We developed a high-throughput assay in a neuronal cell culture model of HD, screened 43,685 compounds and identified 29 novel selective inhibitors of cell death in mutant huntingtin-expressing cells. Four compounds were active in diverse HD models, which suggests a role for cell death in HD; these ...
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Healthy neurons do not store glycogen while they do possess the machinery for the glycogen synthesis albeit at an inactive state. Neurons in the degenerating brain, however, are known to accumulate glycogen, although its significance was not well understood. Emerging reports present contrasting views on neuronal glycogen synthesis; a few reports demonstrate a neurotoxic effect of glycogen while...
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Transgenic mice with a point mutation in the light neurofilament gene develop amyotrophic lateral sclerosis-like motor neuron disease characterized by selective spinal motor neuron loss, neurofilamentous accumulations, and severe muscle atrophy. To test whether the large motor neurons at risk in this disease could be protected from mutant neurofilament-mediated killing, these mice were bred to ...
متن کاملDisassociation of histone deacetylase-3 from normal huntingtin underlies mutant huntingtin neurotoxicity.
Huntington's disease (HD) is caused by a polyglutamine expansion within the huntingtin (Htt) protein. Both loss of function of normal Htt and gain of a toxic function by the polyglutamine-expanded mutant Htt protein have been proposed to be responsible for HD, although the molecular mechanisms involved are unclear. We show that Htt is a neuroprotective protein in both HD-related and unrelated m...
متن کاملHuntington’s Disease: From Mutant Huntingtin Protein to Neurotrophic Factor Therapy
Huntington's disease (HD) is an inherited disorder characterized by neuronal dysfunction and degeneration in striatum and cerebral cortex. Although the signaling pathways involved in HD are not yet clearly elucidated, mutant huntingtin protein is a key factor in the induction of neurodegeneration. The mutant huntingtin protein alters intracellular Ca(2+) homeostasis, disrupts intracellular traf...
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ژورنال
عنوان ژورنال: Cell Death & Differentiation
سال: 2007
ISSN: 1350-9047,1476-5403
DOI: 10.1038/sj.cdd.4402261