Ontology highlight
ABSTRACT:
SUBMITTER: Subramaniam S
PROVIDER: S-EPMC2745286 | biostudies-literature | 2009 Jun
REPOSITORIES: biostudies-literature
Subramaniam Srinivasa S Sixt Katherine M KM Barrow Roxanne R Snyder Solomon H SH
Science (New York, N.Y.) 20090601 5932
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 ...[more]