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Nature and cause of mitochondrial dysfunction in Huntington’s disease: focusing on huntingtin and the striatum

10 Apr 2010

Abstract excerpt

Polyglutamine expansion mutation in huntingtin causes Huntington's disease (HD). How mutant huntingtin (mHtt) preferentially kills striatal neurons remains unknown. The link between mitochondrial dysfunction and HD pathogenesis stemmed from postmortem brain data and mitochondrial toxin models. Current evidence from genetic models, containing mHtt, supports mitochondrial dysfunction with yet uncertain nature and...

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PMID: 20403078
DOI: 10.1111/j.1471-4159.2010.06741.x

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