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| Content Provider | World Health Organization (WHO)-Global Index Medicus |
|---|---|
| Author | Barrow, Roxanne Subramaniam, Srinivasa Snyder, Solomon H. Sixt, Katherine M. |
| Description | Author Affiliation: Subramaniam S ( Solomon H. Snyder Department of Neuroscience, Johns Hopkins University School of Medicine, 725 North Wolfe Street, Baltimore, MD 21205, USA.) |
| 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. |
| ISSN | 00368075 |
| e-ISSN | 10959203 |
| Journal | Science |
| Issue Number | 5932 |
| Volume Number | 324 |
| Language | English |
| Publisher | American Association for the Advancement of Science (United States) |
| Publisher Date | 2009-06-05 |
| Publisher Place | United States |
| Access Restriction | Open |
| Subject Keyword | Cell Death GTP-Binding Proteins Metabolism Nerve Tissue Proteins Nuclear Proteins Animals Cell Line Cell Survival Corpus Striatum Mice Mice, Transgenic Mutant Proteins Chemistry PC12 Cells RNA Interference Recombinant Fusion Proteins SUMO-1 Protein Genetics Small Ubiquitin-Related Modifier Proteins Substrate Specificity Research Support, N.I.H., Extramural Multidisciplinary |
| Content Type | Text |
| Resource Type | Article |
| Subject | History and Philosophy of Science |
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