Study Identifies Promising New Drug Target in Huntington’s Disease; Blocking Function of Striatal-Enriched GTPase Rhes Protein May Halt Initiation of HD

Huntington’s disease attacks the part of the brain that controls movement, destroying nerves with a barrage of toxicity, yet leaves other parts relatively unscathed. Scientists from the Florida campus of The Scripps Research Institute (TSRI) have established conclusively that an activating protein, called “Rhes,” plays a pivotal role in focusing the toxicity of Huntington’s disease in the striatum, a smallish section of the forebrain that controls body movement and is potentially involved in other cognitive functions such as working memory. “Our study definitively confirms the role of Rhes in Huntington’s disease,” said TSRI Assistant Professor Dr. Srinivasa Subramaniam, who led the study. “Our next step should be to develop drugs that inhibit its action.” The study was published online on June 3, 2015 in the journal Neurobiology of Disease. The article is titled ““Ectopic Expression of the Striatal-Enriched GTPase Rhes Elicits Cerebellar Degeneration and an Ataxia Phenotype in Huntington Disease.” In an earlier study, Dr. Subramaniam and his colleagues showed that Rhes binds to a series of repeats in the huntingtin protein (named for its association with Huntington’s disease), increasing the death of neurons. The new study shows that deleting Rhes significantly reduces behavioral problems in animal models of the disease. In addition, the study took the research further and revealed the effects of adding Rhes to the cerebellum, a brain region normally not affected in Huntington’s. Remarkably, Huntington disease animals injected with Rhes experienced an exacerbation of motor issues, including loss of balance and coordination. Dr.
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