Rhes, a striatal specific protein, mediates mutant-huntingtin cytotoxicity.
basic_science · Level V
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- Record sourced from PubMed, PMID 19498170.
- Also identified by DOI 10.1126/science.1172871 and PMC identifier 2745286.
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Abstract
Huntington's disease (HD) is caused by a polyglutamine repeat in the protein huntingtin (Htt) with mutant Htt (mHtt) expressed throughout the body and similarly in all brain regions. Yet, HD neuropathology is largely restricted to the corpus striatum. We report that the small guanine nucleotide-binding protein Rhes, which is localized very selectively to the striatum, binds physiologically to mHtt. Using cultured cells, we found Rhes induces sumoylation of mHtt, which leads to cytotoxicity. Thus, Rhes-mHtt interactions can account for the localized neuropathology of HD.
Medical subject headings
- Cell Death
- GTP-Binding Proteins
- Nerve Tissue Proteins
- Nuclear Proteins