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


RefSeq Accession Definition Service Stock Status Price *Turnaround time Order
NM_001294332 Homo sapiens succinate dehydrogenase complex, subunit A, flavoprotein (Fp) (SDHA), transcript variant 2, mRNA. GenEZ ORF Cloning On-demand $849.00 20
NM_004168 Homo sapiens succinate dehydrogenase complex, subunit A, flavoprotein (Fp) (SDHA), transcript variant 1, mRNA. GenEZ ORF Cloning On-demand $849.00 20
XM_005248329 PREDICTED: Homo sapiens succinate dehydrogenase complex, subunit A, flavoprotein (Fp) (SDHA), transcript variant X1, mRNA. GenEZ ORF Cloning On-demand $849.00 20
XM_005248331 PREDICTED: Homo sapiens succinate dehydrogenase complex, subunit A, flavoprotein (Fp) (SDHA), transcript variant X3, mRNA. GenEZ ORF Cloning On-demand $849.00 20

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Gene Symbol SDHA
Entrez Gene ID 6389
Full Name succinate dehydrogenase complex, subunit A, flavoprotein (Fp)
Synonyms CMD1GG, FP, PGL5, SDH1, SDH2, SDHF
General protein information
Preferred Names
succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial
Names
succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial
flavoprotein subunit of complex II
succinate dehydrogenase complex flavoprotein subunit
NP_004159.2
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. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jun 2014].
Disorder

MIM: 600857

Leigh syndrome, 256000 (3); Mitochondrial respiratory chain complex

mRNA Protein Product Sequence Price Select
NM_001294332, 661567359 NP_001281261, 661567360 succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial isoform 2 ORF Sequence $700.00
NM_004168, 661567358 NP_004159, 156416003 succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial isoform 1 ORF Sequence $700.00
XM_005248329, 530378870 XP_005248386, 530378871 succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial isoform X1 ORF Sequence $700.00
XM_005248331, 530378874 XP_005248388, 530378875 succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial isoform X3 ORF Sequence $700.00
KEGG
hsa00020Citrate cycle (TCA cycle)
hsa00190Oxidative phosphorylation
hsa05016Huntington's disease
hsa05012Parkinson's disease
hsa05010Alzheimer's disease
hsa_M00148Succinate dehydrogenase (ubiquinone)
hsa_M00011Citrate cycle, second carbon oxidation, 2-oxoglutarate => oxaloacetate
hsa01200Carbon metabolism
hsa_M00009Citrate cycle (TCA cycle, Krebs cycle)
hsa04932Non-alcoholic fatty liver disease (NAFLD)
WikiPathways
WP78TCA Cycle
WP111Electron Transport Chain
BIOCYC
HUMAN_PWY-5690TCA cycle II (eukaryotic)
META_PWY66-398TCA cycle VIII (metazoan)
Reactome
REACT_6305Respiratory electron transport, ATP synthesis by chemiosmotic coupling, and heat production by uncoupling proteins.
REACT_1785Citric acid cycle (TCA cycle)
REACT_1046Pyruvate metabolism and Citric Acid (TCA) cycle
REACT_22393Respiratory electron transport
REACT_111217Metabolism
REACT_111083The citric acid (TCA) cycle and respiratory electron transport
Homo sapiens (human)SDHANP_004159.2
Pan troglodytes (chimpanzee)SDHAXP_003310656.1
Macaca mulatta (Rhesus monkey)LOC708018XP_001094170.2
Canis lupus familiaris (dog)SDHAXP_535807.4
Bos taurus (cattle)SDHANP_776603.1
Mus musculus (house mouse)SdhaNP_075770.1
Rattus norvegicus (Norway rat)SdhaNP_569112.1
Gallus gallus (chicken)SDHANP_001264327.1
Danio rerio (zebrafish)sdhaNP_957204.1
Drosophila melanogaster (fruit fly)SdhANP_725882.1
Caenorhabditis eleganssdha-1NP_509446.1
Caenorhabditis eleganssdha-2NP_492798.1
Arabidopsis thaliana (thale cress)SDH1-1NP_201477.1
Xenopus (Silurana) tropicalis (western clawed frog)sdhaNP_001015989.1
Process
IDNameEvidence
GO:0006099tricarboxylic acid cycleIEA
GO:0006099tricarboxylic acid cycleTAS
GO:0006105succinate metabolic processIDA
GO:0007399nervous system developmentIMP
GO:0022904respiratory electron transport chainIDA
GO:0022904respiratory electron transport chainTAS
GO:0044237cellular metabolic processTAS
GO:0044281small molecule metabolic processTAS
GO:0055114oxidation-reduction processIDA
Component
IDNameEvidence
GO:0005739mitochondrionIDA
GO:0005743mitochondrial inner membraneISS
GO:0005743mitochondrial inner membraneTAS
GO:0005749mitochondrial respiratory chain complex IIISS
GO:0005749mitochondrial respiratory chain complex IITAS
Function
IDNameEvidence
GO:0000104succinate dehydrogenase activityIMP
GO:0005515protein bindingIPI
GO:0008177succinate dehydrogenase (ubiquinone) activityIEA
GO:0050660flavin adenine dinucleotide bindingIEA
GeneCards SDHA
UniProt D6RFM5, P31040
Vega OTTHUMG00000090275
MIM 600857
Ensembl ENSG00000073578
HGNC 10680
HPRD 02914

GeneRIFs: Gene References Into Functions What's a GeneRIF?

What is the normal function of the SDHA gene?

The SDHA gene provides instructions for making one of four parts (subunits) of the succinate dehydrogenase (SDH) enzyme. The SDH enzyme plays a critical role in mitochondria, which are structures inside cells that convert the energy from food into a form that cells can use.

Within mitochondria, the SDH enzyme links two important pathways in energy conversion: the citric acid cycle (or Krebs cycle) and oxidative phosphorylation. As part of the citric acid cycle, the SDH enzyme converts a compound called succinate to another compound called fumarate. Negatively charged particles called electrons are released during this reaction. The SDHA protein is the active subunit of the enzyme that performs the conversion of succinate, and it also helps transfer electrons to the oxidative phosphorylation pathway. In oxidative phosphorylation, the electrons help create an electrical charge that provides energy for the production of adenosine triphosphate (ATP), the cell's main energy source.

Succinate, the compound on which the SDH enzyme acts, is an oxygen sensor in the cell and can help turn on specific pathways that stimulate cells to grow in a low-oxygen environment (hypoxia). In particular, succinate stabilizes a protein called hypoxia-inducible factor (HIF) by preventing a reaction that would allow HIF to be broken down. HIF controls several important genes involved in cell division and the formation of new blood vessels in a hypoxic environment.

The SDHA gene is a tumor suppressor gene, which means it prevents cells from growing and dividing in an uncontrolled way.

Interactant

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