Article
Inhibiting the ubiquitin-proteasome system leads to preferential accumulation of toxic N-terminal mutant huntingtin fragments.
Human molecular genetics - 15 Jun 2010
Li Xiang, Wang Chuan-En, Huang Shanshan, Xu Xingshun, Li Xiao-Jiang, Li He, Li Shihua
Abstract excerpt
An expanded polyglutamine (polyQ) domain in the N-terminal region of huntingtin (htt) causes misfolding and accumulation of htt in neuronal cells and the subsequent neurodegeneration of Huntington's disease (HD). Clearing the misfolded htt is critical for preventing neuropathology, and this process is mediated primarily by both the ubiquitin-proteasome system (UPS) and autophagy. Although overexpression of mutant...
Topics
- Animals
- Autophagy
- Brain
- Cell Line
- Gene Knock-In Techniques
- Humans
- Huntingtin Protein
- Huntington Disease
- Mice
- Mutation
- Nerve Tissue Proteins