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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Veröffentlicht in:PLoS One
Hauptverfasser: 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
Format: Artigo
Sprache:Inglês
Veröffentlicht: Public Library of Science 2014
Schlagworte:
Research Article
Online Zugang: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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