Polyglutamine- and Temperature-Dependent Conformational Rigidity in Mutant Huntingtin Revealed by Immunoassays and Circular Dichroism Spectroscopy

BACKGROUND: In Huntington's disease, expansion of a CAG triplet repeat occurs in exon 1 of the huntingtin gene (HTT), resulting in a protein bearing>35 polyglutamine residues whose N-terminal fragments display a high propensity to misfold and aggregate. Recent data demonstrate that polygluta...

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發表在:PLoS One
Main Authors: Fodale, Valentina, Kegulian, Natalie C., Verani, Margherita, Cariulo, Cristina, Azzollini, Lucia, Petricca, Lara, Daldin, Manuel, Boggio, Roberto, Padova, Alessandro, Kuhn, Rainer, Pacifici, Robert, Macdonald, Douglas, Schoenfeld, Ryan C., Park, Hyunsun, Isas, J. Mario, Langen, Ralf, Weiss, Andreas, Caricasole, Andrea
格式: Artigo
語言:Inglês
出版: Public Library of Science 2014
主題:
Research Article
在線閱讀:https://ncbi.nlm.nih.gov/pmc/articles/PMC4251833/
https://ncbi.nlm.nih.gov/pubmed/25464275
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1371/journal.pone.0112262
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