Succinate Dehydrogenase Complex Assembly Factor 2 (SDHAF2)

The SDHAF2 gene encodes a protein necessary for the flavination of the succinate dehydrogenase complex subunit SDHA and thus for SDH activity.The amino acid sequence of yeast Sdh5 is 44% identical from residues 33 to 158 of 163 to its human ortholog. The human SDH5 gene encodes a highly conserved 167-amino acid protein. Yeast Sdh5 localizes to the mitochondrial matrix. Both yeast and human SDH5 interact with the catalytic subunit of the succinate dehydrogenase complex, a component of both the electron transport chain and the tricarboxylic acid cycle. SDH5 is required for SDH-dependent respiration and for flavination (incorporation of the flavin adenine dinucleotide cofactor) of SDH1 (SDHA).