Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial

Details

Name
Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial
Synonyms
  • 1.3.5.1
  • Flavoprotein subunit of complex II
  • Fp
  • SDH2
  • SDHF
Gene Name
SDHA
Organism
Humans
Amino acid sequence
>lcl|BSEQ0000351|Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial
MSGVRGLSRLLSARRLALAKAWPTVLQTGTRGFHFTVDGNKRASAKVSDSISAQYPVVDH
EFDAVVVGAGGAGLRAAFGLSEAGFNTACVTKLFPTRSHTVAAQGGINAALGNMEEDNWR
WHFYDTVKGSDWLGDQDAIHYMTEQAPAAVVELENYGMPFSRTEDGKIYQRAFGGQSLKF
GKGGQAHRCCCVADRTGHSLLHTLYGRSLRYDTSYFVEYFALDLLMENGECRGVIALCIE
DGSIHRIRAKNTVVATGGYGRTYFSCTSAHTSTGDGTAMITRAGLPCQDLEFVQFHPTGI
YGAGCLITEGCRGEGGILINSQGERFMERYAPVAKDLASRDVVSRSMTLEIREGRGCGPE
KDHVYLQLHHLPPEQLATRLPGISETAMIFAGVDVTKEPIPVLPTVHYNMGGIPTNYKGQ
VLRHVNGQDQIVPGLYACGEAACASVHGANRLGANSLLDLVVFGRACALSIEESCRPGDK
VPPIKPNAGEESVMNLDKLRFADGSIRTSELRLSMQKSMQNHAAVFRVGSVLQEGCGKIS
KLYGDLKHLKTFDRGMVWNTDLVETLELQNLMLCALQTIYGAEARKESRGAHAREDYKVR
IDEYDYSKPIQGQQKKPFEEHWRKHTLSYVDVGTGKVTLEYRPVIDKTLNEADCATVPPA
IRSY
Number of residues
664
Molecular Weight
72690.975
Theoretical pI
7.41
GO Classification
Functions
flavin adenine dinucleotide binding / succinate dehydrogenase (ubiquinone) activity / succinate dehydrogenase activity
Processes
cellular metabolic process / nervous system development / oxidation-reduction process / respiratory electron transport chain / small molecule metabolic process / succinate metabolic process / tricarboxylic acid cycle
Components
mitochondrial inner membrane / mitochondrial respiratory chain complex II, succinate dehydrogenase complex (ubiquinone) / mitochondrion / myelin sheath
General Function
Succinate dehydrogenase activity
Specific Function
Flavoprotein (FP) subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q). Can act as a tumor suppressor.
Pfam Domain Function
Transmembrane Regions
Not Available
Cellular Location
Mitochondrion inner membrane
Gene sequence
>lcl|BSEQ0016026|Succinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrial (SDHA)
ATGTCGGGGGTCCGGGGCCTGTCGCGGCTGCTGAGCGCTCGGCGCCTGGCGCTGGCCAAG
GCGTGGCCAACAGTGTTGCAAACAGGAACCCGAGGTTTTCACTTCACTGTTGATGGGAAC
AAGAGGGCATCTGCTAAAGTTTCAGATTCCATTTCTGCTCAGTATCCAGTAGTGGATCAT
GAATTTGATGCAGTGGTGGTAGGCGCTGGAGGGGCAGGCTTGCGAGCTGCATTTGGCCTT
TCTGAGGCAGGGTTTAATACAGCATGTGTTACCAAGCTGTTTCCTACCAGGTCACACACT
GTTGCAGCACAGCTAGAAAATTATGGCATGCCGTTTAGCAGAACTGAAGATGGGAAGATT
TATCAGCGTGCATTTGGTGGACAGAGCCTCAAGTTTGGAAAGGGCGGGCAGGCCCATCGG
TGCTGCTGTGTGGCTGATCGGACTGGCCACTCGCTATTGCACACCTTATATGGAAGGTCT
CTGCGATATGATACCAGCTATTTTGTGGAGTATTTTGCCTTGGATCTCCTGATGGAGAAT
GGGGAGTGCCGTGGTGTCATCGCACTGTGCATAGAGGACGGGTCCATCCATCGCATAAGA
GCAAAGAACACTGTTGTTGCCACAGGAGGCTACGGGCGCACCTACTTCAGCTGCACGTCT
GCCCACACCAGCACTGGCGACGGCACGGCCATGATCACCAGGGCAGGCCTTCCTTGCCAG
GACCTAGAGTTTGTTCAGTTCCACCCTACAGGCATATATGGTGCTGGTTGTCTCATTACG
GAAGGATGTCGTGGAGAGGGAGGCATTCTCATTAACAGTCAAGGCGAAAGGTTTATGGAG
CGATACGCCCCTGTCGCGAAGGACCTGGCGTCTAGAGATGTGGTGTCTCGGTCCATGACT
CTGGAGATCCGAGAAGGAAGAGGCTGTGGCCCTGAGAAAGATCACGTCTACCTGCAGCTG
CACCACCTACCTCCAGAGCAGCTGGCCACGCGCCTGCCTGGCATTTCAGAGACAGCCATG
ATCTTCGCTGGCGTGGACGTCACGAAGGAGCCGATCCCTGTCCTCCCCACCGTGCATTAT
AACATGGGCGGCATTCCCACCAACTACAAGGGGCAGGTCCTGAGGCACGTGAATGGCCAG
GATCAGATTGTGCCCGGCCTGTACGCCTGTGGGGAGGCCGCCTGTGCCTCGGTACATGGT
GCCAACCGCCTCGGGGCAAACTCGCTCTTGGACCTGGTTGTCTTTGGTCGGGCATGTGCC
CTGAGCATCGAAGAGTCATGCAGGCCTGGAGATAAAGTCCCTCCAATTAAACCAAACGCT
GGGGAAGAATCTGTCATGAATCTTGACAAATTGAGATTTGCTGATGGAAGCATAAGAACA
TCGGAACTGCGACTCAGCATGCAGAAGTCAATGCAAAATCATGCTGCCGTGTTCCGTGTG
GGAAGCGTGTTGCAAGAAGGTTGTGGGAAAATCAGCAAGCTCTATGGAGACCTAAAGCAC
CTGAAGACGTTCGACCGGGGAATGGTCTGGAACACGGACCTGGTGGAGACCCTGGAGCTG
CAGAACCTGATGCTGTGTGCGCTGCAGACCATCTACGGAGCAGAGGCACGGAAGGAGTCA
CGGGGCGCGCATGCCAGGGAAGACTACAAGGTGCGGATTGATGAGTACGATTACTCCAAG
CCCATCCAGGGGCAACAGAAGAAGCCCTTTGAGGAGCACTGGAGGAAGCACACCCTGTCC
TATGTGGACGTTGGCACTGGGAAGGTCACTCTGGAATATAGACCCGTGATCGACAAAACT
TTGAACGAGGCTGACTGTGCCACCGTCCCGCCAGCCATTCGCTCCTACTGA
Chromosome Location
5
Locus
5p15
External Identifiers
ResourceLink
UniProtKB IDP31040
UniProtKB Entry NameSDHA_HUMAN
GenBank Protein ID506338
GenBank Gene IDD30648
GenAtlas IDSDHA
HGNC IDHGNC:10680
General References
  1. Hirawake H, Wang H, Kuramochi T, Kojima S, Kita K: Human complex II (succinate-ubiquinone oxidoreductase): cDNA cloning of the flavoprotein (Fp) subunit of liver mitochondria. J Biochem. 1994 Jul;116(1):221-7. [PubMed:7798181]
  2. Morris AA, Farnsworth L, Ackrell BA, Turnbull DM, Birch-Machin MA: The cDNA sequence of the flavoprotein subunit of human heart succinate dehydrogenase. Biochim Biophys Acta. 1994 Mar 29;1185(1):125-8. [PubMed:8142412]
  3. Parfait B, Chretien D, Rotig A, Marsac C, Munnich A, Rustin P: Compound heterozygous mutations in the flavoprotein gene of the respiratory chain complex II in a patient with Leigh syndrome. Hum Genet. 2000 Feb;106(2):236-43. [PubMed:10746566]
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  7. Bourgeron T, Rustin P, Chretien D, Birch-Machin M, Bourgeois M, Viegas-Pequignot E, Munnich A, Rotig A: Mutation of a nuclear succinate dehydrogenase gene results in mitochondrial respiratory chain deficiency. Nat Genet. 1995 Oct;11(2):144-9. [PubMed:7550341]
  8. Aboulaich N, Vainonen JP, Stralfors P, Vener AV: 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. 2004 Oct 15;383(Pt 2):237-48. [PubMed:15242332]
  9. Hao HX, Khalimonchuk O, Schraders M, Dephoure N, Bayley JP, Kunst H, Devilee P, Cremers CW, Schiffman JD, Bentz BG, Gygi SP, Winge DR, Kremer H, Rutter J: SDH5, a gene required for flavination of succinate dehydrogenase, is mutated in paraganglioma. Science. 2009 Aug 28;325(5944):1139-42. doi: 10.1126/science.1175689. Epub 2009 Jul 23. [PubMed:19628817]
  10. Choudhary C, Kumar C, Gnad F, Nielsen ML, Rehman M, Walther TC, Olsen JV, Mann M: Lysine acetylation targets protein complexes and co-regulates major cellular functions. Science. 2009 Aug 14;325(5942):834-40. doi: 10.1126/science.1175371. Epub 2009 Jul 16. [PubMed:19608861]
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Drug Relations

Drug Relations
DrugBank IDNameDrug groupPharmacological action?ActionsDetails
DB00139Succinic acidapproved, nutraceuticalunknownDetails
DB04657Carboxinapproved, experimentalunknownDetails
DB04795ThenoyltrifluoroacetoneexperimentalunknownDetails
DB041412-Hexyloxy-6-Hydroxymethyl-Tetrahydro-Pyran-3,4,5-TriolexperimentalunknownDetails
DB08689UBIQUINONE-1experimentalunknownDetails
DB09270Ubidecarenoneapproved, investigational, nutraceuticalyescofactorDetails
DB13257Ferrous sulfate anhydrousapprovedunknownligandDetails