Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 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

Jared Rutter  1 , Dennis R Winge, Joshua D Schiffman
Affiliations
Review

Succinate dehydrogenase - Assembly, regulation and role in human disease

Jared Rutter et al. Mitochondrion. 2010 Jun.

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.

PubMed Disclaimer

References

    1. Amar L, Bertherat J, et al. Genetic testing in pheochromocytoma or functional paraganglioma. J Clin Oncol. 2005;23(34):8812–8818. - PubMed
    1. Armstrong R, Greenhalgh KL, et al. Succinate dehydrogenase subunit B (SDHB) gene deletion associated with a composite paraganglioma/neuroblastoma. J Med Genet. 2009;46(3):215–216. - PubMed
    1. Astrom K, Cohen JE, et al. Altitude is a phenotypic modifier in hereditary paraganglioma type 1: evidence for an oxygen-sensing defect. Hum Genet. 2003;113(3):228–237. - PubMed
    1. Astuti D, Latif F, et al. Gene mutations in the succinate dehydrogenase subunit SDHB cause susceptibility to familial pheochromocytoma and to familial paraganglioma. Am J Hum Genet. 2001;69(1):49–54. - PMC - PubMed
    1. Astuti D, Morris M, et al. Investigation of the role of SDHB inactivation in sporadic phaeochromocytoma and neuroblastoma. Br J Cancer. 2004;91(10):1835–1841. - PMC - PubMed

MeSH terms

Substances