SDHA

SDHA
Identifiers
Aliases	SDHA, CMD1GG, FP, PGL5, SDH1, SDH2, SDHF, succinate dehydrogenase complex flavoprotein subunit A, MC2DN1, NDAXOA
External IDs	OMIM: 600857; MGI: 1914195; HomoloGene: 3073; GeneCards: SDHA; OMA:SDHA - orthologs
Gene location (Human)
Chr.	Chromosome 5 (human)
End	257,082 bp
Gene location (Mouse)
Chr.	Chromosome 13 (mouse)
End	74,498,399 bp
RNA expression pattern
	Top expressed in
	apex of heart; ; left ventricle; ; mucosa of transverse colon; ; right auricle of heart; ; skeletal muscle tissue; ; muscle of thigh; ; duodenum; ; gastrocnemius muscle; ; right lobe of liver; ; right adrenal cortex;
	Top expressed in
	right ventricle; ; sternocleidomastoid muscle; ; digastric muscle; ; temporal muscle; ; right kidney; ; vastus lateralis muscle; ; triceps brachii muscle; ; aortic valve; ; medial head of gastrocnemius muscle; ; supraoptic nucleus;
	More reference expression data
	n/a
Gene ontology
Molecular function	oxidoreductase activity; oxidoreductase activity, acting on the CH-CH group of donors; succinate dehydrogenase activity; protein binding; succinate dehydrogenase (ubiquinone) activity; flavin adenine dinucleotide binding; electron transfer activity;
Cellular component	mitochondrial inner membrane; myelin sheath; mitochondrial respiratory chain complex II, succinate dehydrogenase complex (ubiquinone); membrane; mitochondrion; nucleolus; plasma membrane succinate dehydrogenase complex;
Biological process	succinate metabolic process; tricarboxylic acid cycle; nervous system development; electron transport chain; respiratory electron transport chain; mitochondrial electron transport, succinate to ubiquinone; anaerobic respiration;
	Sources:Amigo / QuickGO
Orthologs
	6389
	66945
	ENSG00000073578
	ENSMUSG00000021577
	P31040
	Q8K2B3
	NM_001294332; NM_004168; NM_001330758
	NM_023281
	NP_001281261; NP_001317687; NP_004159
	NP_075770
	Wikidata
View/Edit Human	View/Edit Mouse

Succinate dehydrogenase complex, subunit A, flavoprotein variant is a protein that in humans is encoded by the SDHA gene. This gene encodes a major catalytic subunit of succinate-ubiquinone oxidoreductase, a complex of the mitochondrial respiratory chain. The complex is composed of four nuclear-encoded subunits and is localized in the mitochondrial inner membrane. SDHA contains the FAD binding site where succinate is deprotonated and converted to fumarate. Mutations in this gene have been associated with a form of mitochondrial respiratory chain deficiency known as Leigh Syndrome. A pseudogene has been identified on chromosome 3q29. Alternatively spliced transcript variants encoding different isoforms have been found for this gene.