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J Clin Invest. 2011 Feb;121(2):493-9. doi: 10.1172/JCI45691. Epub 2011 Feb 1.

Energy deficit in Huntington disease: why it matters.

Author information

1
INSERM UMR S975, Institut du Cerveau et de la Moelle, Paris, France.

Abstract

Huntington disease (HD) is an autosomal dominant neurodegenerative disease with complete penetrance. Although the understanding of the cellular mechanisms that drive neurodegeneration in HD and account for the characteristic pattern of neuronal vulnerability is incomplete, defects in energy metabolism, particularly mitochondrial function, represent a common thread in studies of HD pathogenesis in humans and animal models. Here we review the clinical, biochemical, and molecular evidence of an energy deficit in HD and discuss the mechanisms underlying mitochondrial and related alterations.

PMID:
21285522
PMCID:
PMC3026743
DOI:
10.1172/JCI45691
[Indexed for MEDLINE]
Free PMC Article

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