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- Title
Energy deficit in Huntington disease: why it matters.
- Authors
Mochel, Fanny; Haller, Ronald G.
- 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.
- Subjects
HUNTINGTON disease; NEURODEGENERATION; DEGENERATION (Pathology); ENERGY metabolism; BIOCHEMISTRY; MICROBIAL respiration
- Publication
Journal of Clinical Investigation, 2011, Vol 121, Issue 2, p493
- ISSN
0021-9738
- Publication type
journal article
- DOI
10.1172/JCI45691