Neurodegeneration: Rhes, SUMOylation, and Huntington's Disease

The small G-protein Rhes (Ras Homolog Enriched in Striatum) is a 266 amino acid protein found predominantly in striatum, and to a lesser extent, the cerebral cortex (Falk et al., 1999).  Recent research has revealed that this GTPase may be key to understanding the paradoxical finding that while many different types of cells throughout the brain and body express wild-type and mutant huntingtin protein (mHTT), striatal neurons (and to a degree, cortical neurons) have a selective vulnerability in Huntington’s disease (HD) (Harrison, 2012; Harrison and LaHoste, 2013).  HD is an autosomal dominant neurodegenerative disorder characterized by >35 CAG repeats in the huntingtin gene, resulting in expression of mHTT that contains multiple glutamine residues (a.k.a. polyglutamine or polyQ repeats).  ... Click to read more

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