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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Publicado en:PLoS One
Autores principales: 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
Formato: Artigo
Lenguaje:Inglês
Publicado: Public Library of Science 2014
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Research Article
Acceso en línea: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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