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We now show a dissociation between huntingtin aggregation and the selective pattern of striatal neuron loss observed in HD. Aggregate formation was predominantly observed in spared interneurons, with few or no aggregates found within vulnerable spiny striatal neurons.
- S Kuemmerle, C A Gutekunst, A M Klein, A M Klein, X J Li, S H Li, M F Beal, M F Beal, S M Hersch, R ...
- 1999
Aug 18, 2024 · Huntington’s disease (HD) is caused by a glutamine repeat expansion in the protein huntingtin. Mutated huntingtin (mHtt) forms aggregates whose impacts on neuronal survival are still...
May 17, 2001 · These findings support the hypothesis that polyglutamine aggregation may not be a predictor of cell loss. Rather than a harbinger of neuronal death, mutant huntingtin aggregation may be a cytoprotective mechanism against polyglutamine-induced neurotoxicity.
- S Kuemmerle, C A Gutekunst, A M Klein, A M Klein, X J Li, S H Li, M F Beal, M F Beal, S M Hersch, R ...
- 1999
Aug 18, 2024 · Huntington's disease (HD) is caused by a glutamine repeat expansion in the protein huntingtin. Mutated huntingtin (mHtt) forms aggregates whose impacts on neuronal survival are still debated. Using weeks-long, continual imaging of cortical neurons, we find that mHtt is gradually sequestrated into pe ….
Aug 4, 2010 · An expanded polyglutamine (polyQ) stretch in the protein huntingtin (htt) induces self-aggregation into inclusion bodies (IBs) and causes Huntington's disease (HD). Defining precise relationships between early observable variables and neuronal death at the molecular and cellular levels should improve our understanding of HD pathogenesis.
- Jason Miller, Montserrat Arrasate, Montserrat Arrasate, Benjamin A. Shaby, Siddhartha Mitra, Eliezer...
- 2010
Miller J, Arrasate M, Shaby BA, Mitra S, Masliah E, Finkbeiner S. Quantitative Relationships between Huntingtin Levels, Polyglutamine Length, Inclusion Body Formation, and Neuronal Death Provide Novel Insight into Huntington’s Disease Molecular Pathogenesis.
Jan 1, 2024 · The aggregates do not correlate with atrophy (Arrasate & Finkbeiner, 2012) while neuronal death can also result from oligomeric stages of aggregation, and there is evidence suggesting that the formation of inclusions may even be protective in animal and cell models (Nucifora et al., 2012).
