Succinate dehydrogenase assembly factor 2 (SDHAF2, also known as Sdh5 or Emi5) is required for insertion of FAD cofactor into SDH1, the catalytic subunit of succinate dehydrogenase (SDH) []. SDH complex is a component of both the electron transport chain and the tricarboxylic acid cycle.This entry includes mitochondrial SDHAF2 from fungi and metazoans. In humans, mutations in SDHAF2 gene cause paragangliomas 2 (PGL2), which is a neural crest tumor usually derived from the chromoreceptor tissue of a paraganglion [].
