Succinate dehydrogenase complex assembly factor 4 (SDHAF4)

Succinate dehydrogenase complex assembly factor 4 (SDHAF4) is a mitochondrial protein essential for the assembly of succinate dehydrogenase (SDH, also known as respiratory complex II), which performs a central role in both the tricarboxylic acid (TCA) cycle and the electron transport chain by coupling the oxidation of succinate to fumarate with reduction of ubiquinone[2][1]. SDHAF4 specifically binds the FAD-containing SDHA subunit, promotes its association with SDHB, and prevents excessive ROS generation by stabilizing assembly intermediates[2][1][3]. Loss of SDHAF4 compromises the activity of SDH, resulting in defects in mitochondrial respiration, accumulation of metabolic intermediates (like succinate), increased oxidative stress, and—depending on tissue context—can contribute to neurodegeneration, muscular dysfunction, and cardiac pathologies[1][3]. SDHAF4 has not been described as a direct therapeutic target or as modulated by approved drugs, but its dysfunction has been implicated in mitochondrial and cardiovascular diseases[2][3].

Other names

Succinate dehydrogenase assembly factor 4, mitochondrialSDH assembly factor 4C6orf57Sdh8UPF0369 protein C6orf57