Article
Mutant huntingtin fragments form oligomers in a polyglutamine length-dependent manner in vitro and in vivo.
The Journal of biological chemistry - 7 May 2010
Legleiter Justin, Mitchell Emily, Lotz Gregor P, Sapp Ellen, Ng Cheping, DiFiglia Marian, Thompson Leslie M, Muchowski Paul J
Abstract excerpt
Huntington disease (HD) is caused by an expansion of more than 35-40 polyglutamine (polyQ) repeats in the huntingtin (htt) protein, resulting in accumulation of inclusion bodies containing fibrillar deposits of mutant htt fragments. Intriguingly, polyQ length is directly proportional to the propensity for htt to form fibrils and the severity of HD and is inversely correlated with age of onset. Although the...
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