TY - JOUR T1 - Gene of the month: SDH JF - Journal of Clinical Pathology JO - J Clin Pathol SP - 95 LP - 97 DO - 10.1136/jclinpath-2017-204677 VL - 71 IS - 2 AU - Alessandro Pietro Aldera AU - Dhirendra Govender Y1 - 2018/02/01 UR - http://jcp.bmj.com/content/71/2/95.abstract N2 - 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. ER -