Article
Polyglutamine- and temperature-dependent conformational rigidity in mutant huntingtin revealed by immunoassays and circular dichroism spectroscopy.
PloS one - 1 Jan 2014
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
Abstract excerpt
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 polyglutamine expansion results in conformational changes in the huntingtin protein (HTT), which likely influence its biological and...
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