Work

Rhes Deletion Is Neuroprotective in the 3-Nitropropionic Acid Model of Huntington's Disease

Year: 2013

Type: article

Abstract: Although the mutated protein causing Huntington's disease (HD) is expressed throughout the body, the major pathology of HD is localized to the striatum of the brain. We previously reported that the striatal-enriched protein Rhes binds the mutated huntingtin protein and enhances its cytotoxicity. We now demonstrate that Rhes-deleted mice are dramatically protected from neurotoxicity and motor dysfunction in a striatal-specific model of HD elicited by 3-nitropropionic acid. This finding suggests that Rhes may, in part, determine the striatal selectivity of HD.

Source: Journal of Neuroscience

DOI: 10.1523/jneurosci.3730-12.2013

Authors: Robert G. Mealer, Srinivasa Subramaniam, Solomon H. Snyder

Institutions: Johns Hopkins University, Johns Hopkins Medicine

Language: English

Topic: Genetic Neurodegenerative Diseases

Subfield: Cellular and Molecular Neuroscience

Field: Neuroscience

Domain: Life Sciences

SDG: Good health and well-being

Open Access status: hybrid

Funders: U.S. Public Health Service, National Institute of Mental Health