NameSuccinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial
Synonyms
  • CII-4
  • CybS
  • QPs3
  • SDH4
  • Succinate dehydrogenase complex subunit D
  • Succinate-ubiquinone oxidoreductase cytochrome b small subunit
  • Succinate-ubiquinone reductase membrane anchor subunit
Gene NameSDHD
OrganismHuman
Amino acid sequence
>lcl|BSEQ0004420|Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial
MAVLWRLSAVCGALGGRALLLRTPVVRPAHISAFLQDRPIPEWCGVQHIHLSPSHHSGSK
AASLHWTSERVVSVLLLGLLPAAYLNPCSAMDYSLAAALTLHGHWGLGQVVTDYVHGDAL
QKAAKAGLLALSALTFAGLCYFNYHDVGICKAVAMLWKL
Number of residues159
Molecular Weight17042.82
Theoretical pI8.75
GO Classification
Functions
  • ubiquinone binding
  • heme binding
  • metal ion binding
  • succinate dehydrogenase activity
  • electron carrier activity
Processes
  • cellular metabolic process
  • respiratory electron transport chain
  • tricarboxylic acid cycle
  • small molecule metabolic process
Components
  • mitochondrial respiratory chain complex II, succinate dehydrogenase complex (ubiquinone)
  • mitochondrion
  • mitochondrial inner membrane
  • mitochondrial envelope
  • integral component of membrane
General FunctionUbiquinone binding
Specific FunctionMembrane-anchoring 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).
Pfam Domain Function
Transmembrane Regions64-85 91-111 121-142
GenBank Protein ID2351037
UniProtKB IDO14521
UniProtKB Entry NameDHSD_HUMAN
Cellular LocationMitochondrion inner membrane
Gene sequence
>lcl|BSEQ0011623|Succinate dehydrogenase [ubiquinone] cytochrome b small subunit, mitochondrial (SDHD)
ATGGCGGTTCTCTGGAGGCTGAGTGCCGTTTGCGGTGCCCTAGGAGGCCGAGCTCTGTTG
CTTCGAACTCCAGTGGTCAGACCTGCTCATATCTCAGCATTTCTTCAGGACCGACCTATC
CCAGAATGGTGTGGAGTGCAGCACATACACTTGTCACCGAGCCACCATTGGGCCTTGGAC
AAGTTGTTACTGACTATGTTCATGGGGATGCCTTGCAGAAAGCTGCCAAGGCAGGGCTTT
TGGCACTTTCAGCTTTAA
GenBank Gene IDAB006202
GeneCard IDNot Available
GenAtlas IDSDHD
HGNC IDHGNC:10683
Chromosome Location11
Locus11q23
References
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  2. Hirawake H, Taniwaki M, Tamura A, Amino H, Tomitsuka E, Kita K: Characterization of the human SDHD gene encoding the small subunit of cytochrome b (cybS) in mitochondrial succinate-ubiquinone oxidoreductase. Biochim Biophys Acta. 1999 Aug 4;1412(3):295-300. 10482792
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  25. Alston CL, Ceccatelli Berti C, Blakely EL, Olahova M, He L, McMahon CJ, Olpin SE, Hargreaves IP, Nolli C, McFarland R, Goffrini P, O'Sullivan MJ, Taylor RW: A recessive homozygous p.Asp92Gly SDHD mutation causes prenatal cardiomyopathy and a severe mitochondrial complex II deficiency. Hum Genet. 2015 Aug;134(8):869-79. doi: 10.1007/s00439-015-1568-z. Epub 2015 May 26. 26008905