SDHA

SDHA
Identifiers
Aliases	SDHA, CMD1GG, FP, PGL5, SDH1, SDH2, SDHF, succinate dehydrogenase complex flavoprotein subunit A
External IDs	OMIM: 600857 MGI: 1914195 HomoloGene: 3073 GeneCards: SDHA
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 carrier 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 • oxidation-reduction process • nervous system development • electron transport chain • respiratory electron transport chain • mitochondrial electron transport, succinate to ubiquinone • anaerobic respiration Sources:Amigo / QuickGO
Orthologs
Species	Human	Mouse
Entrez	6389	66945
Ensembl	ENSG00000073578	ENSMUSG00000021577
UniProt	P31040	Q8K2B3
RefSeq (mRNA)	NM_001294332 NM_004168 NM_001330758	NM_023281
RefSeq (protein)	NP_001281261 NP_001317687 NP_004159	NP_075770.1 NP_075770
Location (UCSC)	Chr 5: 0.22 – 0.26 Mb	Chr 13: 74.32 – 74.35 Mb
PubMed search

6389

66945

ENSG00000073578

ENSMUSG00000021577

P31040

Q8K2B3

NM_001294332
NM_004168
NM_001330758

NM_023281

NP_001281261
NP_001317687
NP_004159

NP_075770.1
NP_075770

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.

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