NameCytochrome b-c1 complex subunit Rieske, mitochondrial
Synonyms
  • 1.10.2.2
  • Complex III subunit 5
  • Cytochrome b-c1 complex subunit 5
  • Rieske iron-sulfur protein
  • RISP
  • Ubiquinol-cytochrome c reductase iron-sulfur subunit
Gene NameUQCRFS1
OrganismHuman
Amino acid sequence
>lcl|BSEQ0012690|Cytochrome b-c1 complex subunit Rieske, mitochondrial
MLSVASRSGPFAPVLSATSRGVAGALRPLVQATVPATPEQPVLDLKRPFLSRESLSGQAV
RRPLVASVGLNVPASVCYSHTDIKVPDFSEYRRLEVLDSTKSSRESSEARKGFSYLVTGV
TTVGVAYAAKNAVTQFVSSMSASADVLALAKIEIKLSDIPEGKNMAFKWRGKPLFVRHRT
QKEIEQEAAVELSQLRDPQHDLDRVKKPEWVILIGVCTHLGCVPIANAGDFGGYYCPCHG
SHYDASGRIRLGPAPLNLEVPTYEFTSDDMVIVG
Number of residues274
Molecular Weight29667.695
Theoretical pI8.46
GO Classification
Functions
  • metal ion binding
  • ubiquinol-cytochrome-c reductase activity
  • 2 iron, 2 sulfur cluster binding
Processes
  • response to drug
  • response to antibiotic
  • small molecule metabolic process
  • cellular metabolic process
  • respiratory electron transport chain
  • response to hormone
Components
  • myelin sheath
  • mitochondrial respiratory chain complex III
  • mitochondrion
  • mitochondrial inner membrane
General FunctionUbiquinol-cytochrome-c reductase activity
Specific FunctionComponent of the ubiquinol-cytochrome c reductase complex (complex III or cytochrome b-c1 complex), which is a respiratory chain that generates an electrochemical potential coupled to ATP synthesis.The transit peptide of the Rieske protein seems to form part of the bc1 complex and is considered to be the subunit 11/IX of that complex.
Pfam Domain Function
Transmembrane Regions103-140
GenBank Protein ID158255704
UniProtKB IDP47985
UniProtKB Entry NameUCRI_HUMAN
Cellular LocationMitochondrion inner membrane
Gene sequence
>lcl|BSEQ0012691|Cytochrome b-c1 complex subunit Rieske, mitochondrial (UQCRFS1)
ATGTTGTCGGTAGCATCCCGCTCAGGCCCGTTCGCGCCCGTCCTGTCGGCCACGTCCCGC
GGGGTGGCGGGCGCGCTGCGGCCCTTGGTGCAGGCCACGGTGCCCGCCACCCCGGAGCAG
CCTGTGTTGGACCTGAAGCGGCCCTTCCTCAGCCGGGAGTCGCTGAGCGGCCAGGCCGTG
CGCCGGCCTTTGGTCGCCTCCGTGGGCCTCAATGTCCCTGCTTCTGTTTGTTATTCCCAC
ACAGACATCAAGGTGCCTGACTTCTCTGAATACCGCCGCCTTGAAGTTTTAGATAGTACG
AAGTCTTCAAGAGAAAGCAGCGAGGCTAGGAAAGGTTTCTCCTATTTGGTAACTGGAGTA
ACTACTGTGGGTGTCGCATATGCTGCCAAGAATGCCGTCACCCAGTTCGTTTCCAGCATG
AGTGCTTCTGCTGATGTGTTGGCCCTGGCGAAAATCGAAATCAAGTTATCCGATATTCCA
GAAGGCAAGAACATGGCTTTCAAATGGAGAGGCAAACCCCTGTTTGTGCGTCATAGAACC
CAGAAGGAAATTGAGCAGGAAGCTGCAGTTGAATTATCACAGTTGAGGGACCCACAGCAT
GATCTAGATCGAGTAAAGAAACCTGAATGGGTTATCCTGATAGGTGTTTGCACTCATCTT
GGCTGTGTACCCATTGCAAATGCAGGAGATTTTGGTGGTTATTACTGCCCTTGCCATGGG
TCACACTATGATGCATCTGGCAGGATCAGATTGGGTCCTGCTCCTCTCAACCTTGAAGTC
CCCACGTATGAGTTCACCAGTGACGATATGGTGATTGTTGGTTAA
GenBank Gene IDAK291134
GeneCard IDNot Available
GenAtlas IDNot Available
HGNC IDHGNC:12587
Chromosome Location19
Locus19q12-q13.1
References
  1. Nishikimi M, Hosokawa Y, Toda H, Suzuki H, Ozawa T: The primary structure of human Rieske iron-sulfur protein of mitochondrial cytochrome bc1 complex deduced from cDNA analysis. Biochem Int. 1990;20(1):155-60. 2158323
  2. Pennacchio LA, Bergmann A, Fukushima A, Okubo K, Salemi A, Lennon GG: Structure, sequence and location of the UQCRFS1 gene for the human Rieske Fe-S protein. Gene. 1995 Apr 3;155(2):207-11. 7721092
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