Mutant Huntingtin Fragments Form Oligomers in a Polyglutamine Length-dependent Manner in Vitro and in Vivo

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 propensi...

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Autors principals: Legleiter, Justin, Mitchell, Emily, Lotz, Gregor P., Sapp, Ellen, Ng, Cheping, DiFiglia, Marian, Thompson, Leslie M., Muchowski, Paul J.
Format: Artigo
Idioma:Inglês
Publicat: American Society for Biochemistry and Molecular Biology 2010
Matèries:
Protein Structure and Folding
Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC2863238/
https://ncbi.nlm.nih.gov/pubmed/20220138
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1074/jbc.M109.093708
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