Succinate dehydrogenase assembly factor 1, mitochondrial (SDH assembly factor 1) (SDHAF1) (LYR motif-containing protein 8)
1_MSRHS 6_ RLQRQ 11_ VLSLY 16_ RDLLR 21_ AGRGK 26_ PGAEA 31_ RVRAE 36_ FRQHA 41_ GLPRS 46_ DVLRI 51_ EYLYR 56_ RGRRQ 61_ LQLLR 66_ SGHAT 71_ AMGAF 76_ VRPRA 81_ PTGEP 86_ GGVGC 91_ QPDDG 96_ DSPRN 101_ PHDST 106_GAPET
1: Plays an essential role in the assembly of succinate dehydrogenase (SDH), an enzyme complex (also referred to as respiratory complex II) that is a component of both the tricarboxylic acid (TCA) cycle and the mitochondrial electron transport chain, and which couples the oxidation of succinate to fumarate with the reduction of ubiquinone (coenzyme Q) to ubiquinol (PubMed:19465911, PubMed:24954417). Promotes maturation of the iron-sulfur protein subunit SDHB of the SDH catalytic dimer, protecting it from the deleterious effects of oxidants (PubMed:24954417). May act together with SDHAF3 (PubMed:24954417). Contributes to iron-sulfur cluster incorporation into SDHB by binding to SDHB and recruiting the iron-sulfur transfer complex formed by HSC20, HSPA9 and ISCU through direct binding to HSC20 (PubMed:26749241)