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Gene of the month: SDH
  1. Alessandro Pietro Aldera,
  2. Dhirendra Govender
  1. Division of Anatomical Pathology, Faculty of Health Sciences, University of Cape Town and National Health Laboratory Service – Groote Schuur hospital, Cape Town, South Africa
  1. Correspondence to Professor Dhirendra Govender, Division of Anatomical Pathology, Faculty of Health Sciences, University of Cape Town, Cape Town 7925, South Africa; dhiren.govender{at}


Succinate dehydrogenase (SDH) is a heterotetrameric nuclear encoded mitochondrial protein complex which plays a role in the citric acid cycle and the electron transfer chain. Germline mutations in SDHA are associated with Leigh syndrome. Mutations in SDHB, SDHC and SDHD are found in an increasing number of neoplasms, most notably paragangliomas and wild-type gastrointestinal stromal tumours. SDH deficiency in these tumours has important prognostic implications, and also provides a novel target for molecular therapy. In this article, we outline the structure and function of SDH and provide a summary of its role in various diseases.

  • genetics
  • neoplasms
  • molecular pathology

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  • Handling editor Tahir S Pillay.

  • Contributors Both authors have contributed equally to the manuscript.

  • Competing interests None declared.

  • Provenance and peer review Commissioned; internally peer reviewed.