Uncontrolled movements are symptomatic of Huntington's disease, which is caused by mutation of the huntingtin gene. Mutant huntingtin protein (mHtt) is expressed throughout the bodies of carriers, but mHtt-associated cell death occurs selectively in certain brain regions, including the striatum, which is important for movement.
A team led by Solomon Snyder at Johns Hopkins University School of Medicine in Baltimore, Maryland, found that Rhes, a protein located mainly in the striatum, binds to mHtt and attaches a small protein to it. This modification increased cell death in cultured cells, suggesting that Rhes interacts with mHtt to cause localized neurodegeneration.
Rights and permissions
About this article
Cite this article
Neurogenetics: Huntington's toxic trigger. Nature 459, 754–755 (2009). https://doi.org/10.1038/459754f
Published:
Issue Date:
DOI: https://doi.org/10.1038/459754f
