Journal article
A patient-derived cellular model for Huntington’s disease reveals phenotypes at clinically relevant CAG lengths
Abstract
The huntingtin protein participates in several cellular processes that are disrupted when the polyglutamine tract is expanded beyond a threshold of 37 CAG DNA repeats in Huntington's disease (HD). Cellular biology approaches to understand these functional disruptions in HD have primarily focused on cell lines with synthetically long CAG length alleles that clinically represent outliers in this disease and a more severe form of HD that lacks age …
Authors
Hung CL-K; Maiuri T; Bowie LE; Gotesman R; Son S; Falcone M; Giordano JV; Gillis T; Mattis V; Lau T
Journal
Molecular Biology of the Cell, Vol. 29, No. 23, pp. 2809–2820
Publisher
American Society for Cell Biology (ASCB)
Publication Date
November 15, 2018
DOI
10.1091/mbc.e18-09-0590
ISSN
1059-1524