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SDHA succinate dehydrogenase complex, subunit A, flavoprotein (Fp) [Homo sapiens (human)]


RefSeq Accession Definition Sequence Price Select
NM_004168 Homo sapiens succinate dehydrogenase complex, subunit A, flavoprotein (Fp) (SDHA), nuclear gene encoding mitochondrial protein, mRNA. Full Length $697.45
ORF Sequence $578.55


Gene Symbol SDHA
Entrez Gene ID 6389
Full Name succinate dehydrogenase complex, subunit A, flavoprotein (Fp)
Synonyms CMD1GG, FP, SDH1, SDH2, SDHF
Gene Type protein-coding
Organism Homo sapiens (human)
Genome

5

5p15
Summary 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. 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. [provided by RefSeq].
Disorder

MIM: 600857

Leigh syndrome, 256000 (3); Mitochondrial respiratory chain complex
mRNA NM_004168, 156416002
Protein NP_004159, 156416003
Product succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial precursor
RefSeq Status REVIEWED
KEGG
hsa00020Citrate cycle (TCA cycle)
hsa05016Huntington's disease
hsa05012Parkinson's disease
hsa01100Metabolic pathways
hsa05010Alzheimer's disease
hsa00190Oxidative phosphorylation
WikiPathways
WP78TCA Cycle
WP111Electron Transport Chain
BIOCYC
META_PWY-5690TCA cycle variation III (eukaryotic)
Reactome
REACT_1046Pyruvate metabolism and Citric Acid (TCA) cycle
REACT_1785Citric acid cycle (TCA cycle)
REACT_22393Respiratory electron transport
REACT_6305Respiratory electron transport, ATP synthesis by chemiosmotic coupling, and heat production by uncoupling proteins.
Homo sapiens (human)SDHANP_004159.2
Canis lupus familiaris (dog)SDHAXP_856901.1
Mus musculus (house mouse)SdhaNP_075770.1
Rattus norvegicus (Norway rat)SdhaNP_569112.1
Gallus gallus (chicken)SDHAXP_419054.2
Danio rerio (zebrafish)sdhaNP_957204.1
Drosophila melanogaster (fruit fly)Scs-fpNP_725881.1
Caenorhabditis eleganssdha-1NP_509446.1
Caenorhabditis eleganssdha-2NP_492798.1
Arabidopsis thaliana (thale cress)SDH1-2NP_179435.1
Process
IDNameEvidence
GO:0006099tricarboxylic acid cycleTAS
GO:0006105succinate metabolic processIDA
GO:0006810transportIEA
GO:0007399nervous system developmentIMP
GO:0022904respiratory electron transport chainIDA
GO:0022904respiratory electron transport chainTAS
GO:0055114oxidation-reduction processIDA
Component
IDNameEvidence
GO:0005739mitochondrionIDA
GO:0005743mitochondrial inner membraneEXP
GO:0005743mitochondrial inner membraneISS
GO:0005749mitochondrial respiratory chain complex IIISS
GO:0005749mitochondrial respiratory chain complex IITAS
GO:0016020membraneIEA
Function
IDNameEvidence
GO:0000104succinate dehydrogenase activityIMP
GO:0005515protein bindingIPI
GO:0008177succinate dehydrogenase (ubiquinone) activityIEA
GO:0009055electron carrier activityIEA
GO:0050660flavin adenine dinucleotide bindingIEA
GeneCards SDHA
UniProt P31040
MIM 600857
Ensembl ENSG00000073578
HGNC 10680
HPRD 02914

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