SDHAF1 (Succinate dehydrogenase complex assembly factor 1) is one of the subunits of the succinate dehydrogenase (SDH) complex (or complex II) of the mitochondrial respiratory chain. SDHAF1 resides in the mitochondria, and is essential for SDH assembly, but does not physically associate with the complex in vivo. Mutations in this gene are associated with SDH-defective infantile leukoencephalopathy (mitochondrial complex II deficiency). Unlike the related protein SDHAF2, SDHAF1 is not thought to be a tumor suppressor.Synonyms: LYR motif-containing protein 8, SDH assembly factor 1, Succinate dehydrogenase assembly factor 1 mitochondrial