Venue: Edificio Povo 2, via Sommarive nr. 9, Povo (Tn) - Room B105
at 2:00 p.m.
- Srinivasa Subramaniam - The Scripps Research Institute-Florida, Jupiter, Florida, USA
Huntington’s disease (HD) is a genetic disorder with early, prominent involvement of the striatum, a region that controls motor and cognitive function. HD is provoked by an expansion of glutamine repeats in the huntingtin protein (Htt), leading to mutant Htt (mHtt), which is widely expressed throughout the brain and peripheral tissues, yet predominantly leads to the damage and death of striatal neurons. The molecular basis for this specificity is unclear, and etiology-based therapies for this devastating disease remain elusive. Therefore, studies that define the mechanisms that contribute to this disease are critical to develop new drugs that prevent and/or delay the onset of HD. Our goal is to understand the role of striatal-specific proteins in HD pathogenesis for preventive and therapeutic purposes. We have identified Rhes (Ras–homolog enriched in striatum)–mTORC1 as a major signaling circuitry that elicits striatal abnormalities in HD. I will discuss some of the experimental evidences for this Rhes-mTORC1 circuitry in HD.