NameCytochrome c1, heme protein, mitochondrial
Synonyms
  • Complex III subunit 4
  • Complex III subunit IV
  • Cytochrome b-c1 complex subunit 4
  • Cytochrome c-1
  • Ubiquinol-cytochrome-c reductase complex cytochrome c1 subunit
Gene NameCYC1
OrganismHuman
Amino acid sequence
>lcl|BSEQ0020718|Cytochrome c1, heme protein, mitochondrial
MAAAAASLRGVVLGPRGAGLPGARARGLLCSARPGQLPLRTPQAVALSSKSGLSRGRKVM
LSALGMLAAGGAGLAMALHSAVSASDLELHPPSYPWSHRGLLSSLDHTSIRRGFQVYKQV
CASCHSMDFVAYRHLVGVCYTEDEAKELAAEVEVQDGPNEDGEMFMRPGKLFDYFPKPYP
NSEAARAANNGALPPDLSYIVRARHGGEDYVFSLLTGYCEPPTGVSLREGLYFNPYFPGQ
AIAMAPPIYTDVLEFDDGTPATMSQIAKDVCTFLRWASEPEHDHRKRMGLKMLMMMALLV
PLVYTIKRHKWSVLKSRKLAYRPPK
Number of residues325
Molecular Weight35421.61
Theoretical pI9.25
GO Classification
Functions
  • metal ion binding
  • electron transporter, transferring electrons from CoQH2-cytochrome c reductase complex and cytochrome c oxidase complex activity
  • heme binding
Processes
  • small molecule metabolic process
  • cellular metabolic process
  • respiratory electron transport chain
  • response to glucagon
Components
  • mitochondrial inner membrane
  • integral component of membrane
  • respiratory chain
  • mitochondrion
  • membrane
  • nucleus
General FunctionMetal ion binding
Specific FunctionThis is the heme-containing component of the cytochrome b-c1 complex, which accepts electrons from Rieske protein and transfers electrons to cytochrome c in the mitochondrial respiratory chain.
Pfam Domain Function
Transmembrane Regions292-306
GenBank Protein ID181238
UniProtKB IDP08574
UniProtKB Entry NameCY1_HUMAN
Cellular LocationMitochondrion inner membrane
Gene sequence
>lcl|BSEQ0020719|Cytochrome c1, heme protein, mitochondrial (CYC1)
ATGGCGGCAGCTGCGGCTTCGCTTCGCGGGGTAGTGTTGGGCCCGCGGGGCGCGGGGCTC
CCGGGCGCGCGTGCCCGGGGTCTGCTGTGCAGCGCGCGTCCCGGGCAGCTCCCGCTACGG
ACACCTCAGGCAGTGGCCTTGTCGTCGAAGTCTGGCCTTTCCCGAGGCCGGAAAGTGATG
CTGTCAGCGCTGGGCATGCTGGCGGCAGGGGGTGCGGGGCTGGCCGTGGCTCTGCATTCG
GCTGTGAGTGCCAGTGACCTGGAGCTGCACCCCCCCAGCTATCCGTGGTCTCACCGTGGC
CTCCTCTCTTCCTTGGACCACACCAGCATCCGGAGGGGTTTCCAGGTATATAAGCAGGTG
TGCGCCTCCTGCCACAGCATGGACTTCGTGGCCTACCGCCACCTGGTGGGCGTGTGCTAC
ACGGAGGATGAAGCTAAGGAGCTGGCTGCGGAGGTGGAGGTTCAAGACGGCCCCAATGAA
GATGGGGAGATGTTCATGCGGCCAGGGAAGCTGTTCGACTATTTCCCAAAACCATACCCC
AACAGTGAGGCTGCTCGAGCTGCCAACAACGGAGCATTGCCCCCTGACCTCAGCTACATC
GTGCGAGCTAGGCATGGTGGTGAGGACTACGTCTTCTCCCTGCTCACGGGCTACTGCGAG
CCACCCACCGGGGTGTCACTGCGGGAAGGTCTCTACTTCAACCCCTACTTTCCTGGCCAG
GCCATTGCCATGGCCCCTCCCATCTACACAGATGTCTTAGAGTTTGACGATGGCACCCCA
GCTACCATGTCCCAGATAGCCAAGGATGTGTGCACCTTCCTGCGCTGGGCATCTGAGCCA
GAGCACGACCATCGAAAACGCATGGGGCTCAAGATGTTGATGATGATGGCTCTGCTGGTG
CCCCTGGTCTACACCATAAAGCGGCACAAGTGGTCAGTCCTGAAGAGTCGGAAGCTGGCA
TATCGGCCGCCCAAGTGA
GenBank Gene IDM16597
GeneCard IDNot Available
GenAtlas IDNot Available
HGNC IDHGNC:2579
Chromosome Location8
Locus8q24.3
References
  1. Nishikimi M, Ohta S, Suzuki H, Tanaka T, Kikkawa F, Tanaka M, Kagawa Y, Ozawa T: Nucleotide sequence of a cDNA encoding the precursor to human cytochrome c1. Nucleic Acids Res. 1988 Apr 25;16(8):3577. 2836796
  2. Suzuki H, Hosokawa Y, Nishikimi M, Ozawa T: Structural organization of the human mitochondrial cytochrome c1 gene. J Biol Chem. 1989 Jan 25;264(3):1368-74. 2536365
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  5. Nishikimi M, Suzuki H, Ohta S, Sakurai T, Shimomura Y, Tanaka M, Kagawa Y, Ozawa T: Isolation of a cDNA clone for human cytochrome c1 from a lambda gt11 expression library. Biochem Biophys Res Commun. 1987 May 29;145(1):34-9. 3036122
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  8. Vaca Jacome AS, Rabilloud T, Schaeffer-Reiss C, Rompais M, Ayoub D, Lane L, Bairoch A, Van Dorsselaer A, Carapito C: N-terminome analysis of the human mitochondrial proteome. Proteomics. 2015 Jul;15(14):2519-24. doi: 10.1002/pmic.201400617. Epub 2015 Jun 8. 25944712
  9. Valnot I, Kassis J, Chretien D, de Lonlay P, Parfait B, Munnich A, Kachaner J, Rustin P, Rotig A: A mitochondrial cytochrome b mutation but no mutations of nuclearly encoded subunits in ubiquinol cytochrome c reductase (complex III) deficiency. Hum Genet. 1999 Jun;104(6):460-6. 10453733
  10. Gaignard P, Menezes M, Schiff M, Bayot A, Rak M, Ogier de Baulny H, Su CH, Gilleron M, Lombes A, Abida H, Tzagoloff A, Riley L, Cooper ST, Mina K, Sivadorai P, Davis MR, Allcock RJ, Kresoje N, Laing NG, Thorburn DR, Slama A, Christodoulou J, Rustin P: Mutations in CYC1, encoding cytochrome c1 subunit of respiratory chain complex III, cause insulin-responsive hyperglycemia. Am J Hum Genet. 2013 Aug 8;93(2):384-9. doi: 10.1016/j.ajhg.2013.06.015. Epub 2013 Aug 1. 23910460