Energy deficit in Huntington disease: Why it matters

Fanny Mochel, Ronald G. Haller

Research output: Contribution to journalReview article

123 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)493-499
Number of pages7
JournalJournal of Clinical Investigation
Volume121
Issue number2
DOIs
StatePublished - Feb 1 2011

ASJC Scopus subject areas

  • Medicine(all)

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