SDHA Recombinant Rabbit Monoclonal Antibody [JM10-83] (ET1703-40)

UNIPROT #

P31040

PROTEIN NAME

SDHA

SYNONYMS

CMD1GG antibody;DHSA_HUMAN antibody;Flavoprotein subunit of complex II antibody;Fp antibody;PGL5 antibody;SDH 2 antibody;SDH1 antibody;SDH2 antibody;SDHA antibody;SDHF antibody;Succinate dehydrogenase [ubiquinone] flavoprotein subunit antibody;Succinate dehydrogenase [ubiquinone] flavoprotein subunit mitochondrial antibody;Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial antibody;Succinate dehydrogenase complex flavoprotein subunit A antibody;Succinate dehydrogenase complex flavoprotein subunit antibody;Succinate dehydrogenase complex flavoprotein subunit precursor antibody;Succinate dehydrogenase complex subunit A antibody;Succinate dehydrogenase complex subunit A flavoprotein (Fp) antibody;Succinate dehydrogenase complex subunit A flavoprotein antibody

SEQUENCE SIMILARITIES

Belongs to the FAD-dependent oxidoreductase 2 family. FRD/SDH subfamily.

POST-TRANSLATIONAL MODIFICATION

Phosphorylation at Tyr-215 is important for efficient electron transfer in complex II and the prevention of ROS generation.; Acetylated. Deacetylated by SIRT3.

SUBCELLULAR LOCATION

Mitochondrion inner membrane.

FUNCTION

In aerobic respiration reactions, succinate dehydrogenase (SDH) catalyzes the oxidation of succinate and ubiquinone to fumarate and ubiquinol. Four subunits comprise the SDH protein complex: a flavochrome subunit (SDHA), an iron-sulfur protein (SDHB), and two membrane-bound subunits (SDHC and SDHD) anchored to the inner mitochondrial membrane. Mutations to these subunits cause mitochondrial dysfunction, corresponding to several distinct disorders. Mutations in the membrane bound components may cause hereditary paraganglioma, while SDHA mutations are associated with juvenile encephalopathy as well as Leigh Syndrome, a severe neurological disorder. Inactivating mutations in SDHB correlate with inherited, but not necessarily sporadic, cases of pheochromocytoma.