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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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Foilsithe in: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
Formáid: Artigo
Teanga:Inglês
Foilsithe: Public Library of Science 2014
Ábhair:
Rochtain Ar Líne: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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