Article
Sensitive biochemical aggregate detection reveals aggregation onset before symptom development in cellular and murine models of Huntington's disease.
Journal of neurochemistry - 1 Feb 2008
Weiss Andreas, Klein Corinna, Woodman Ben, Sathasivam Kirupa, Bibel Miriam, Régulier Etienne, Bates Gillian P, Paganetti Paolo
Abstract excerpt
A CAG-repeat gene expansion translated into a pathogenic polyglutamine stretch at the N-terminus of huntingtin triggers Huntington's Disease. Mutated huntingtin is predicted to adopt toxic properties mainly if aggregation-prone N-terminal fragments are released by proteolysis. Huntingtin-aggregates are indeed a major hallmark of this disorder and could represent useful markers of disease-onset or progression. We...
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