NameNADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial
Synonyms
  • CI-13kD-A
  • Complex I-13kD-A
  • NADH-ubiquinone oxidoreductase 13 kDa-A subunit
Gene NameNDUFS6
OrganismHuman
Amino acid sequence
>lcl|BSEQ0016013|NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial
MAAAMTFCRLLNRCGEAARSLPLGARCFGVRVSPTGEKVTHTGQVYDDKDYRRIRFVGRQ
KEVNENFAIDLIAEQPVSEVETRVIACDGGGGALGHPKVYINLDKETKTGTCGYCGLQFR
QHHH
Number of residues124
Molecular Weight13711.535
Theoretical pI8.38
GO Classification
Functions
  • electron carrier activity
  • NADH dehydrogenase (ubiquinone) activity
Processes
  • mitochondrial electron transport, NADH to ubiquinone
  • small molecule metabolic process
  • cellular metabolic process
  • multicellular organism growth
  • respiratory electron transport chain
  • muscle contraction
  • fatty acid metabolic process
  • cardiovascular system development
  • multicellular organismal aging
  • mitochondrion morphogenesis
  • reproductive system development
Components
  • mitochondrial respiratory chain complex I
  • mitochondrial inner membrane
General FunctionNadh dehydrogenase (ubiquinone) activity
Specific FunctionAccessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone.
Pfam Domain Function
Transmembrane RegionsNot Available
GenBank Protein ID3348137
UniProtKB IDO75380
UniProtKB Entry NameNDUS6_HUMAN
Cellular LocationMitochondrion inner membrane
Gene sequence
>lcl|BSEQ0016014|NADH dehydrogenase [ubiquinone] iron-sulfur protein 6, mitochondrial (NDUFS6)
ATGGCGGCGGCGATGACCTTCTGCCGGCTGCTGAACCGGTGTGGCGAGGCGGCGCGGAGC
CTGCCCCTGGGCGCCAGGTGTTTCGGGGTGCGGGTCTCGCCGACCGGGGAGAAGGTCACG
CACACTGGCCAGGTTTATGATGATAAAGACTACAGGAGAATTCGGTTTGTAGGTCGTCAG
AAAGAGGTGAATGAAAACTTTGCCATTGATTTGATAGCAGAGCAGCCCGTGAGCGAGGTG
GAGACTCGGGTGATAGCGTGCGATGGCGGCGGGGGAGCTCTTGGCCACCCAAAAGTGTAT
ATAAACTTGGACAAAGAAACAAAAACCGGCACATGCGGTTACTGTGGGCTCCAGTTCAGA
CAGCACCACCACTAG
GenBank Gene IDAF044959
GeneCard IDNot Available
GenAtlas IDNDUFS6
HGNC IDHGNC:7713
Chromosome Location5
Locus5p15.33
References
  1. Loeffen J, van den Heuvel L, Smeets R, Triepels R, Sengers R, Trijbels F, Smeitink J: cDNA sequence and chromosomal localization of the remaining three human nuclear encoded iron sulphur protein (IP) subunits of complex I: the human IP fraction is completed. Biochem Biophys Res Commun. 1998 Jun 29;247(3):751-8. 9647766
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  3. Murray J, Zhang B, Taylor SW, Oglesbee D, Fahy E, Marusich MF, Ghosh SS, Capaldi RA: The subunit composition of the human NADH dehydrogenase obtained by rapid one-step immunopurification. J Biol Chem. 2003 Apr 18;278(16):13619-22. Epub 2003 Feb 28. 12611891
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