2021
DOI: 10.1101/2021.06.16.448681
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Rhes Deletion Prevents Age-Dependent Selective Motor Deficits and Reduces Phosphorylation of S6K in Huntington Disease Hdh150Q(CAG) Knock-In Mice

Abstract: Huntington disease (HD) is a neurodegenerative disease caused by a CAG trinucleotide repeat expansion in the huntingtin (mHTT) protein. This expansion is thought to promote striatal atrophy by a combination of cell- and non-cell-autonomous processes, but the mechanisms are unclear. Previous evidence suggests that the striatal-enriched SUMO E3-like protein Rhes could play a pathological role in HD. Rhes interacts with, and SUMOylates, mHTT and promotes toxicity and Rhes deletion ameliorates the HD phenotype in … Show more

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Cited by 2 publications
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“…Surprisingly, the SUMO E3-like protein Rhes levels are down-regulated in the Hdh150Q knockin mouse striatum ( 64 ), indicating that compensatory mechanisms are at work. Perhaps clearance of Rhes will decrease the SUMOylated forms of mHTT and extend the onset and reduce the severity of the disease process.…”
Section: Discussionmentioning
confidence: 99%
“…Surprisingly, the SUMO E3-like protein Rhes levels are down-regulated in the Hdh150Q knockin mouse striatum ( 64 ), indicating that compensatory mechanisms are at work. Perhaps clearance of Rhes will decrease the SUMOylated forms of mHTT and extend the onset and reduce the severity of the disease process.…”
Section: Discussionmentioning
confidence: 99%