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Article

Possible involvement of self-defense mechanisms in the preferential vulnerability of the striatum in Huntington's disease

26 Sept 2014

Abstract excerpt

HD is caused by a mutation in the huntingtin gene that consists in a CAG repeat expansion translated into an abnormal poly-glutamine (polyQ) tract in the huntingtin (Htt) protein. The most striking neuropathological finding in HD is the atrophy of the striatum. The regional expression of mutant Htt (mHtt) is ubiquitous in the brain and cannot explain by itself the preferential vulnerability of the striatum in HD....

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PMID: 25309327
DOI: 10.3389/fncel.2014.00295

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