Article
Spontaneous self-assembly of pathogenic huntingtin exon 1 protein into amyloid structures.
Essays in biochemistry - 1 Jan 2014
Trepte Philipp, Strempel Nadine, Wanker Erich E
Abstract excerpt
PolyQ (polyglutamine) diseases such as HD (Huntington's disease) or SCA1 (spinocerebellar ataxia type 1) are neurodegenerative disorders caused by abnormally elongated polyQ tracts in human proteins. PolyQ expansions promote misfolding and aggregation of disease-causing proteins, leading to the appearance of nuclear and cytoplasmic inclusion bodies in patient neurons. Several lines of experimental evidence...
Read the complete abstract on PubMedTopics
Share this publication in a Topic to start or enrich a Post.
