Article
Chromosomal instability during neurogenesis in Huntington's disease.
Development (Cambridge, England) - 29 Jan 2018
Ruzo Albert, Croft Gist F, Metzger Jakob J, Galgoczi Szilvia, Gerber Lauren J, Pellegrini Cecilia, Wang Hanbin, Fenner Maria, Tse Stephanie, Marks Adam, Nchako Corbyn, Brivanlou Ali H
Abstract excerpt
Huntington's disease (HD) is a fatal neurodegenerative disease caused by expansion of CAG repeats in the Huntingtin gene (HTT). Neither its pathogenic mechanisms nor the normal functions of HTT are well understood. To model HD in humans, we engineered a genetic allelic series of isogenic human embryonic stem cell (hESC) lines with graded increases in CAG repeat length. Neural differentiation of these lines...
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