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PMID:19498170

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Citation

Subramaniam, S, Sixt, KM, Barrow, R and Snyder, SH (2009) Rhes, a striatal specific protein, mediates mutant-huntingtin cytotoxicity. Science 324:1327-30

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.

Links

PubMed PMC2745286 Online version:10.1126/science.1172871

Keywords

Animals; Cell Death; Cell Line; Cell Survival; Corpus Striatum/metabolism; GTP-Binding Proteins/metabolism; Humans; Mice; Mice, Transgenic; Mutant Proteins/metabolism; Nerve Tissue Proteins/chemistry; Nerve Tissue Proteins/metabolism; Nuclear Proteins/chemistry; Nuclear Proteins/metabolism; PC12 Cells; RNA Interference; Rats; Recombinant Fusion Proteins/metabolism; SUMO-1 Protein/genetics; SUMO-1 Protein/metabolism; Small Ubiquitin-Related Modifier Proteins/metabolism; Substrate Specificity

Significance

Annotations

Gene product Qualifier GO Term Evidence Code with/from Aspect Extension Notes Status

HUMAN:HD

enables

GO:0005515: protein binding

ECO:0000353: physical interaction evidence used in manual assertion

UniProtKB:P63033

F

Seeded From UniProt

complete

RAT:RHES

enables

GO:0005515: protein binding

ECO:0000353: physical interaction evidence used in manual assertion

UniProtKB:P42858

F

Seeded From UniProt

complete

RAT:RHES

enables

GO:0031624: ubiquitin conjugating enzyme binding

ECO:0000353: physical interaction evidence used in manual assertion

UniProtKB:P63281

F

Seeded From UniProt

complete

RAT:RHES

involved_in

GO:0033235: positive regulation of protein sumoylation

ECO:0000314: direct assay evidence used in manual assertion

P

Seeded From UniProt

complete

RAT:RHES

involved_in

GO:0031397: negative regulation of protein ubiquitination

ECO:0000314: direct assay evidence used in manual assertion

P

Seeded From UniProt

complete

RAT:UBC9

enables

GO:0005515: protein binding

ECO:0000353: physical interaction evidence used in manual assertion

UniProtKB:P63033

F

Seeded From UniProt

complete

See also

References

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