Concepedia

Polyglutamine- and Temperature-Dependent Conformational Rigidity in Mutant Huntingtin Revealed by Immunoassays and Circular Dichroism Spectroscopy

Valentina Fodale, Natalie C. Kegulian, Margherita Verani, Cristina Cariulo, Lucia Azzollini, Lara Petricca, Manuel Daldin, Roberto Boggio, Alessandro Padova, Rainer Kühn,

PLoS ONE · 2014 · 48 citations · 62 references

DOIFull text

Open access

Abstract

The temperature- and polyglutamine-dependent effects observed with TR-FRET on HTT proteins represent a simple, scalable, quantitative and sensitive assay to identify genetic and pharmacological modulators of mutant HTT conformation, and potentially to assess the relevance of conformational changes during onset and progression of Huntington's disease.

References

62

A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes

Marcy E. MacDonald · Cell · 1993 · 8.3K citations · Full text

, , +10

A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. The Huntington's Disease Collaborative Research Group.

Manish A. Shah, Nicole A. Datson, Lakshmi Srinidhi et al. · Cell · 1993 · 3.1K citations

, , +10

Aggregation of Huntingtin in Neuronal Intranuclear Inclusions and Dystrophic Neurites in Brain

Marian DiFiglia, Ellen Sapp, Kathryn Chase et al. · Science · 1997 · 2.9K citations

DICHROWEB, an online server for protein secondary structure analyses from circular dichroism spectroscopic data

Lee Whitmore, B.A. Wallace · Nucleic Acids Research · 2004 · 2.3K citations · Full text

, , +25

Histone deacetylase inhibitors arrest polyglutamine-dependent neurodegeneration in Drosophila

Joan S. Steffan, László Bodai, Judit Pallos et al. · Nature · 2001 · 1.2K citations

, , +6