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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Bibliographische Detailangaben
Hauptverfasser: Legleiter, Justin, Mitchell, Emily, Lotz, Gregor P., Sapp, Ellen, Ng, Cheping, DiFiglia, Marian, Thompson, Leslie M., Muchowski, Paul J.
Format: Artigo
Sprache:Inglês
Veröffentlicht: American Society for Biochemistry and Molecular Biology 2010
Schlagworte:
Protein Structure and Folding
Online Zugang: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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