Succinate Dehydrogenase Complex Subunit A (SDHA)

SDH2; SDHF; FP; Flavoprotein; Flavoprotein subunit of complex II; Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial

SPN	N981
HGNC	10680
MGI	1914195
PubMed	23174939
RGD	621557
UniProt	Q59GW8
Wiki	SDHA

SDHA 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.

Hirawake et al. (1994) isolated a cDNA corresponding to the flavoprotein subunit of SDH from a human liver cDNA library. The deduced 621-amino acid protein has a molecular mass of approximately 68 kD and shows high homology to the bovine heart flavoprotein subunit.

Reference

"Human complex II (succinate-ubiquinone oxidoreductase): cDNA cloning of the flavoprotein (Fp) subunit of liver mitochondria."
J. Biochem. 116:221-227(1994) [PubMed] [Europe PMC] [Abstract]

"The cDNA sequence of the flavoprotein subunit of human heart succinate dehydrogenase."
Biochim. Biophys. Acta 1185:125-128(1994) [PubMed] [Europe PMC] [Abstract]

"Compound heterozygous mutations in the flavoprotein gene of the respiratory chain complex II in a patient with Leigh syndrome."
Hum. Genet. 106:236-243(2000) [PubMed] [Europe PMC] [Abstract]

"Complete sequencing and characterization of 21,243 full-length human cDNAs."
Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]

"The DNA sequence and comparative analysis of human chromosome 5."
Nature 431:268-274(2004) [PubMed] [Europe PMC] [Abstract]

"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]

"Cloning of the flavoprotein subunit of human succinate dehydrogenase."
(In) Curti B., Ronchi S., Zanetti G. (eds.);Flavins and flavoproteins 1990, pp.727-730, Walter de Gruyter, Berlin (1991)

"Mutation of a nuclear succinate dehydrogenase gene results in mitochondrial respiratory chain deficiency."
Nat. Genet. 11:144-149(1995) [PubMed] [Europe PMC] [Abstract]

"Vectorial proteomics reveal targeting, phosphorylation and specific fragmentation of polymerase I and transcript release factor (PTRF) at the surface of caveolae in human adipocytes."
Biochem. J. 383:237-248(2004) [PubMed] [Europe PMC] [Abstract]

"SDH5, a gene required for flavination of succinate dehydrogenase, is mutated in paraganglioma."
Science 325:1139-1142(2009) [PubMed] [Europe PMC] [Abstract]

"Lysine acetylation targets protein complexes and co-regulates major cellular functions."
Science 325:834-840(2009) [PubMed] [Europe PMC] [Abstract]

"SDHA is a tumor suppressor gene causing paraganglioma."
Hum. Mol. Genet. 19:3011-3020(2010) [PubMed] [Europe PMC] [Abstract]

"Initial characterization of the human central proteome."
BMC Syst. Biol. 5:17-17(2011) [PubMed] [Europe PMC] [Abstract]

"Mitochondrial c-Src regulates cell survival through phosphorylation of respiratory chain components."
Biochem. J. 447:281-289(2012) [PubMed] [Europe PMC] [Abstract]

"The mitochondrial chaperone TRAP1 promotes neoplastic growth by inhibiting succinate dehydrogenase."
Cell Metab. 17:988-999(2013) [PubMed] [Europe PMC] [Abstract]

"An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."
J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]

"Homozygous Gly555Glu mutation in the nuclear-encoded 70 kDa flavoprotein gene causes instability of the respiratory chain complex II."
Am. J. Med. Genet. A 120:13-18(2003) [PubMed] [Europe PMC] [Abstract]

"Familial neonatal isolated cardiomyopathy caused by a mutation in the flavoprotein subunit of succinate dehydrogenase."
Eur. J. Hum. Genet. 18:1160-1165(2010) [PubMed] [Europe PMC] [Abstract]