Article
Huntington's disease cerebrospinal fluid seeds aggregation of mutant huntingtin.
Molecular psychiatry - 1 Nov 2015
Tan Z, Dai W, van Erp T G M, Overman J, Demuro A, Digman M A, Hatami A, Albay R, Sontag E M, Potkin K T, Ling S, Macciardi F, Bunney W E, Long J D, Paulsen J S, Ringman J M, Parker I, Glabe C, Thompson L M, Chiu W, Potkin S G
Abstract excerpt
Huntington's disease (HD), a progressive neurodegenerative disease, is caused by an expanded CAG triplet repeat producing a mutant huntingtin protein (mHTT) with a polyglutamine-repeat expansion. Onset of symptoms in mutant huntingtin gene-carrying individuals remains unpredictable. We report that synthetic polyglutamine oligomers and cerebrospinal fluid (CSF) from BACHD transgenic rats and from human HD subjects...
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