Article
Identification of novel potentially toxic oligomers formed in vitro from mammalian-derived expanded huntingtin exon-1 protein.
The Journal of biological chemistry - 4 May 2012
Nucifora Leslie G, Burke Kathleen A, Feng Xia, Arbez Nicolas, Zhu Shanshan, Miller Jason, Yang Guocheng, Ratovitski Tamara, Delannoy Michael, Muchowski Paul J, Finkbeiner Steven, Legleiter Justin, Ross Christopher A, Poirier Michelle A
Abstract excerpt
Huntington disease is a genetic neurodegenerative disorder that arises from an expanded polyglutamine region in the N terminus of the HD gene product, huntingtin. Protein inclusions comprised of N-terminal fragments of mutant huntingtin are a characteristic feature of disease, though are likely to play a protective role rather than a causative one in neurodegeneration. Soluble oligomeric assemblies of huntingtin...
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