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Mitochondrion. 2010 Jun;10(4):393-401. doi: 10.1016/j.mito.2010.03.001. Epub 2010 Mar 10.

Succinate dehydrogenase - Assembly, regulation and role in human disease.

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1
Departments of Biochemistry, University of Utah School of Medicine, Salt Lake City, UT, United States. rutter@biochem.utah.edu

Abstract

Succinate dehydrogenase (or Electron Transport Chain Complex II) has been the subject of a focused but significant renaissance. This complex, which has been the least studied of the mitochondrial respiratory complexes has seen renewed interest due to the discovery of its role in human disease. Under this heightened scrutiny, the succinate dehydrogenase complex has proven to be a fascinating machine, whose regulation and assembly requires additional factors that are beginning to be discovered. Mutations in these factors and in the structural subunits of the complex itself cause a variety of human diseases. The mechanisms underlying the pathogenesis of SDH mutations is beginning to be understood.

PMID:
20226277
PMCID:
PMC2874626
DOI:
10.1016/j.mito.2010.03.001
[Indexed for MEDLINE]
Free PMC Article
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