Article
Mutant huntingtin directly increases susceptibility of mitochondria to the calcium-induced permeability transition and cytochrome c release.
Human molecular genetics - 15 Jul 2004
Choo Yeun Su, Johnson Gail V W, MacDonald Marcy, Detloff Peter J, Lesort Mathieu
Abstract excerpt
Huntington's disease (HD) is initiated by an abnormally expanded polyglutamine stretch in the huntingtin protein, conferring a novel property on the protein that leads to the loss of striatal neurons. Defects in mitochondrial function have been implicated in the pathogenesis of HD. Here, we have examined the hypothesis that the mutant huntingtin protein may directly interact with the mitochondrion and affect its...
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