Mitochondrial tumour suppressors: a genetic and biochemical update

Nat Rev Cancer. 2005 Nov;5(11):857-66. doi: 10.1038/nrc1737.


Since the discovery 5 years ago that the D-subunit of succinate dehydrogenase (SDHD) can behave as a classic tumour suppressor, other nuclear-encoded mitochondrial proteins (SDHB, SDHC and fumarate hydratase) have been implicated in tumour susceptibility. Mutations in these proteins are principally involved in familial predisposition to benign tumours, but the spectrum of inherited lesions is increasingly recognized to include malignant tumours, such as malignant phaeochromocytomas and renal cell carcinomas. Here we review recent advances in the field of mitochondrial tumour suppressors, the biochemical pathway that links mitochondrial dysfunction with tumorigenesis, and potential therapeutic approaches to these malignancies.

Publication types

  • Review

MeSH terms

  • Animals
  • Genes, Tumor Suppressor*
  • Genetic Predisposition to Disease*
  • Humans
  • Mitochondria / physiology*
  • Mutation
  • Neoplasms / genetics*
  • Tumor Suppressor Proteins / genetics


  • Tumor Suppressor Proteins