Succinate dehydrogenase - Assembly, regulation and role in human disease

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


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.

Publication types

  • Review

MeSH terms

  • Gene Expression Regulation*
  • Genetic Diseases, Inborn / enzymology*
  • Humans
  • Mitochondrial Proteins / biosynthesis*
  • Mitochondrial Proteins / chemistry
  • Mitochondrial Proteins / deficiency*
  • Succinate Dehydrogenase / biosynthesis*
  • Succinate Dehydrogenase / chemistry
  • Succinate Dehydrogenase / deficiency*


  • Mitochondrial Proteins
  • Succinate Dehydrogenase