Polyglutamine- and temperature-dependent conformational rigidity in mutant huntingtin revealed by immunoassays and circular dichroism spectroscopy.

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 polyglutam...

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Bibliographic Details
Main Authors: Valentina Fodale, Natalie C Kegulian, Margherita Verani, Cristina Cariulo, Lucia Azzollini, Lara Petricca, Manuel Daldin, Roberto Boggio, Alessandro Padova, Rainer Kuhn, Robert Pacifici, Douglas Macdonald, Ryan C Schoenfeld, Hyunsun Park, J Mario Isas, Ralf Langen, Andreas Weiss, Andrea Caricasole
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2014-01-01
Series:PLoS ONE
Online Access:http://europepmc.org/articles/PMC4251833?pdf=render

Internet

http://europepmc.org/articles/PMC4251833?pdf=render

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