Version 5.4
Homo sapiens Gene: SDHA
Summary
InnateDB Gene IDBG-10076.6
Last Modified 2014-10-13 [Report errors or provide feedback]
Gene Symbol SDHA
Gene Name succinate dehydrogenase complex, subunit A, flavoprotein (Fp)
Synonyms CMD1GG; FP; PGL5; SDH1; SDH2; SDHF
Species Homo sapiens
Ensembl Gene ENSG00000073578
Encoded Proteins
IDBP-10082
succinate dehydrogenase complex, subunit A, flavoprotein (Fp)
IDBP-482753
succinate dehydrogenase complex, subunit A, flavoprotein (Fp)
IDBP-478100
succinate dehydrogenase complex, subunit A, flavoprotein (Fp)
IDBP-483387
succinate dehydrogenase complex, subunit A, flavoprotein (Fp)
IDBP-481817
succinate dehydrogenase complex, subunit A, flavoprotein (Fp)
IDBP-484959
succinate dehydrogenase complex, subunit A, flavoprotein (Fp)
IDBP-810920
succinate dehydrogenase complex, subunit A, flavoprotein (Fp)
Protein Structure
Useful resources Stemformatics EHFPI ImmGen
Entrez Gene
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, Jul 2008]
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]
Gene Information
Type Protein coding
Genomic Location Chromosome 5:218241-256700
Strand Forward strand
Band p15.33
Transcripts
ENST00000264932 ENSP00000264932
ENST00000504309 ENSP00000426514
ENST00000502379
ENST00000505555
ENST00000510361 ENSP00000427703
ENST00000509632 ENSP00000425077
ENST00000504824
ENST00000509420
ENST00000514027
ENST00000514233
ENST00000512962
ENST00000515752
ENST00000511810
ENST00000515815 ENSP00000422404
ENST00000509082
ENST00000503674
ENST00000507266
ENST00000509564 ENSP00000421911
ENST00000507522
ENST00000617470 ENSP00000484230
Interactions
Number of Interactions This gene and/or its encoded proteins are associated with 67 experimentally validated interaction(s) in this database.
They are also associated with 1 interaction(s) predicted by orthology.
Experimentally validated
Total 67 [view]
Protein-Protein 67 [view]
Protein-DNA 0
Protein-RNA 0
DNA-DNA 0
RNA-RNA 0
DNA-RNA 0
Predicted by orthology
Total 1 [view]
Gene Ontology

Molecular Function
Accession GO Term
GO:0000104 succinate dehydrogenase activity
GO:0005515 protein binding
GO:0008177 succinate dehydrogenase (ubiquinone) activity
GO:0016491 oxidoreductase activity
GO:0016627 oxidoreductase activity, acting on the CH-CH group of donors
GO:0050660 flavin adenine dinucleotide binding
Biological Process
GO:0006099 tricarboxylic acid cycle
GO:0006105 succinate metabolic process
GO:0007399 nervous system development
GO:0022900 electron transport chain
GO:0022904 respiratory electron transport chain
GO:0044237 cellular metabolic process
GO:0044281 small molecule metabolic process
GO:0055114 oxidation-reduction process
Cellular Component
GO:0005739 mitochondrion
GO:0005743 mitochondrial inner membrane
GO:0005749 mitochondrial respiratory chain complex II
Orthologs
Species
Mus musculus
Bos taurus
Gene ID
Gene Order
ENSMUSG00000021577
View Gene Order
ENSBTAG00000046019
Not yet available
Pathways
NETPATH
REACTOME
REACT_22393
Respiratory electron transport pathway
REACT_1785
Citric acid cycle (TCA cycle) pathway
REACT_1046
Pyruvate metabolism and Citric Acid (TCA) cycle pathway
REACT_6305
Respiratory electron transport, ATP synthesis by chemiosmotic coupling, and heat production by uncoupling proteins. pathway
REACT_111083
The citric acid (TCA) cycle and respiratory electron transport pathway
REACT_111217
Metabolism pathway
KEGG
hsa00020
Citrate cycle (TCA cycle) pathway
hsa05010
Alzheimer's disease pathway
hsa00190
Oxidative phosphorylation pathway
hsa05012
Parkinson's disease pathway
hsa05016
Huntington's disease pathway
INOH
INOH website
Arginine Proline metabolism pathway
INOH website
Citrate cycle pathway
INOH website
Tyrosine metabolism pathway
PID NCI
Cross-References
SwissProt
TrEMBL
UniProt Splice Variant
Entrez Gene
UniGene Hs.440475 Hs.607328
RefSeq NM_001294332 NM_004168 XM_005248331
HUGO
OMIM
CCDS CCDS3853
HPRD 02914
IMGT
EMBL
GenPept
RNA Seq Atlas

If you use InnateDB for your research, please cite the following publication:

Breuer et al., InnateDB: systems biology of innate immunity and beyond - recent updates and continuing curation. Nucl. Acids Res. (2013) 41 (D1) 

InnateDB is being developed jointly by the Brinkman Laboratory (Simon Fraser University, British Columbia, Canada), the Hancock Laboratory (University of British Columbia, Vancouver, British Columbia) and the Lynn EMBL Australia Group (South Australian Health & Medical Research Institute and Flinders University, Adelaide, Australia).

Funding is currently provided by Allergen and EMBL Australia. Previous funding has been provided by Genome Canada, the Foundation for the National Institutes of Health through the Grand Challenges in Global Health initiative and by Teagasc. InnateDB curated interactions are licensed under the Design Science License. All other data is licensed under the terms of the originating database. Contact: innatedb-mail@sfu.ca