Article
Correlative light and electron microscopy suggests that mutant huntingtin dysregulates the endolysosomal pathway in presymptomatic Huntington's disease.
Acta neuropathologica communications - 14 Apr 2021
Zhou Ya, Peskett Thomas R, Landles Christian, Warner John B, Sathasivam Kirupa, Smith Edward J, Chen Shu, Wetzel Ronald, Lashuel Hilal A, Bates Gillian P, Saibil Helen R
Abstract excerpt
Huntington's disease (HD) is a late onset, inherited neurodegenerative disorder for which early pathogenic events remain poorly understood. Here we show that mutant exon 1 HTT proteins are recruited to a subset of cytoplasmic aggregates in the cell bodies of neurons in brain sections from presymptomatic HD, but not wild-type, mice. This occurred in a disease stage and polyglutamine-length dependent manner. We...
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