Hmdb loader
Identification
HMDB Protein ID HMDBP00990
Secondary Accession Numbers
  • 6278
Name Cytochrome b-c1 complex subunit Rieske, mitochondrial
Synonyms
  1. Complex III subunit 5
  2. Complex III subunit IX
  3. Cytochrome b-c1 complex subunit 11
  4. Cytochrome b-c1 complex subunit 5
  5. RISP
  6. Rieske iron-sulfur protein
  7. Ubiquinol-cytochrome c reductase 8 kDa protein
  8. Ubiquinol-cytochrome c reductase iron-sulfur subunit
Gene Name UQCRFS1
Protein Type Enzyme
Biological Properties
General Function Involved in oxidoreductase activity
Specific Function Component 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 (By similarity).
Pathways
  • Alzheimer disease
  • Cardiac muscle contraction
  • Huntington disease
  • Oxidative phosphorylation
  • Parkinson disease
Reactions
QH(2) + ferricytochrome c → Q + ferrocytochrome c + Hydrogen Ion details
GO Classification
Biological Process
small molecule metabolic process
response to hormone stimulus
response to drug
response to antibiotic
respiratory electron transport chain
Cellular Component
mitochondrial respiratory chain complex III
integral to membrane
Component
membrane
cell part
Function
binding
catalytic activity
metal cluster binding
iron-sulfur cluster binding
transmembrane transporter activity
substrate-specific transmembrane transporter activity
ion transmembrane transporter activity
cation transmembrane transporter activity
inorganic cation transmembrane transporter activity
monovalent inorganic cation transmembrane transporter activity
hydrogen ion transmembrane transporter activity
ubiquinol-cytochrome-c reductase activity
oxidoreductase activity, acting on diphenols and related substances as donors
2 iron, 2 sulfur cluster binding
oxidoreductase activity
transporter activity
Molecular Function
metal ion binding
2 iron, 2 sulfur cluster binding
ubiquinol-cytochrome-c reductase activity
Process
metabolic process
oxidation reduction
Cellular Location
  1. Mitochondrion inner membrane
Gene Properties
Chromosome Location 19
Locus 19q12
SNPs UQCRFS1
Gene Sequence
>825 bp
ATGTTGTCGGTAGCATCCCGCTCAGGCCCGTTCGCGCCCGTCCTGTCGGCCACGTCCCGC
GGGGTGGCGGGCGCGCTGCGGCCCTTGGTGCAGGCCACGGTGCCCGCCACCCCGGAGCAG
CCTGTGTTGGACCTGAAGCGGCCCTTCCTCAGCCGGGAGTCGCTGAGCGGCCAGGCCGTG
CGCCGGCCTTTGGTCGCCTCCGTGGGCCTCAATGTCCCTGCTTCTGTTTGTTATTCCCAC
ACAGACATCAAGGTGCCTGACTTCTCTGAATACCGCCGCCTTGAAGTTTTAGATAGTACG
AAGTCTTCAAGAGAAAGCAGCGAGGCTAGGAAAGGTTTCTCCTATTTGGTAACTGGAGTA
ACTACTGTGGGTGTCGCATATGCTGCCAAGAATGCCGTCACCCAGTTCGTTTCCAGCATG
AGTGCTTCTGCTGATGTGTTGGCCCTGGCGAAAATCGAAATCAAGTTATCCGATATTCCA
GAAGGCAAGAACATGGCTTTCAAATGGAGAGGCAAACCCCTGTTTGTGCGTCATAGAACC
CAGAAGGAAATTGAGCAGGAAGCTGCAGTTGAATTATCACAGTTGAGGGACCCACAGCAT
GATCTAGATCGAGTAAAGAAACCTGAATGGGTTATCCTGATAGGTGTTTGCACTCATCTT
GGCTGTGTACCCATTGCAAATGCAGGAGATTTTGGTGGTTATTACTGCCCTTGCCATGGG
TCACACTATGATGCATCTGGCAGGATCAGATTGGGTCCTGCTCCTCTCAACCTTGAAGTC
CCCACGTATGAGTTCACCAGTGACGATATGGTGATTGTTGGTTAA
Protein Properties
Number of Residues 274
Molecular Weight 29667.695
Theoretical pI 8.319
Pfam Domain Function
Signals Not Available
Transmembrane Regions Not Available
Protein Sequence
>Cytochrome b-c1 complex subunit Rieske, mitochondrial
MLSVASRSGPFAPVLSATSRGVAGALRPLVQATVPATPEQPVLDLKRPFLSRESLSGQAV
RRPLVASVGLNVPASVCYSHTDIKVPDFSEYRRLEVLDSTKSSRESSEARKGFSYLVTGV
TTVGVAYAAKNAVTQFVSSMSASADVLALAKIEIKLSDIPEGKNMAFKWRGKPLFVRHRT
QKEIEQEAAVELSQLRDPQHDLDRVKKPEWVILIGVCTHLGCVPIANAGDFGGYYCPCHG
SHYDASGRIRLGPAPLNLEVPTYEFTSDDMVIVG
GenBank ID Protein 5042402
UniProtKB/Swiss-Prot ID P47985
UniProtKB/Swiss-Prot Entry Name UCRI_HUMAN
PDB IDs Not Available
GenBank Gene ID AC007786
GeneCard ID UQCRFS1
GenAtlas ID UQCRFS1
HGNC ID HGNC:12587
References
General References
  1. Ota T, Suzuki Y, Nishikawa T, Otsuki T, Sugiyama T, Irie R, Wakamatsu A, Hayashi K, Sato H, Nagai K, Kimura K, Makita H, Sekine M, Obayashi M, Nishi T, Shibahara T, Tanaka T, Ishii S, Yamamoto J, Saito K, Kawai Y, Isono Y, Nakamura Y, Nagahari K, Murakami K, Yasuda T, Iwayanagi T, Wagatsuma M, Shiratori A, Sudo H, Hosoiri T, Kaku Y, Kodaira H, Kondo H, Sugawara M, Takahashi M, Kanda K, Yokoi T, Furuya T, Kikkawa E, Omura Y, Abe K, Kamihara K, Katsuta N, Sato K, Tanikawa M, Yamazaki M, Ninomiya K, Ishibashi T, Yamashita H, Murakawa K, Fujimori K, Tanai H, Kimata M, Watanabe M, Hiraoka S, Chiba Y, Ishida S, Ono Y, Takiguchi S, Watanabe S, Yosida M, Hotuta T, Kusano J, Kanehori K, Takahashi-Fujii A, Hara H, Tanase TO, Nomura Y, Togiya S, Komai F, Hara R, Takeuchi K, Arita M, Imose N, Musashino K, Yuuki H, Oshima A, Sasaki N, Aotsuka S, Yoshikawa Y, Matsunawa H, Ichihara T, Shiohata N, Sano S, Moriya S, Momiyama H, Satoh N, Takami S, Terashima Y, Suzuki O, Nakagawa S, Senoh A, Mizoguchi H, Goto Y, Shimizu F, Wakebe H, Hishigaki H, Watanabe T, Sugiyama A, Takemoto M, Kawakami B, Yamazaki M, Watanabe K, Kumagai A, Itakura S, Fukuzumi Y, Fujimori Y, Komiyama M, Tashiro H, Tanigami A, Fujiwara T, Ono T, Yamada K, Fujii Y, Ozaki K, Hirao M, Ohmori Y, Kawabata A, Hikiji T, Kobatake N, Inagaki H, Ikema Y, Okamoto S, Okitani R, Kawakami T, Noguchi S, Itoh T, Shigeta K, Senba T, Matsumura K, Nakajima Y, Mizuno T, Morinaga M, Sasaki M, Togashi T, Oyama M, Hata H, Watanabe M, Komatsu T, Mizushima-Sugano J, Satoh T, Shirai Y, Takahashi Y, Nakagawa K, Okumura K, Nagase T, Nomura N, Kikuchi H, Masuho Y, Yamashita R, Nakai K, Yada T, Nakamura Y, Ohara O, Isogai T, Sugano S: Complete sequencing and characterization of 21,243 full-length human cDNAs. Nat Genet. 2004 Jan;36(1):40-5. Epub 2003 Dec 21. [PubMed:14702039 ]
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  3. Grimwood J, Gordon LA, Olsen A, Terry A, Schmutz J, Lamerdin J, Hellsten U, Goodstein D, Couronne O, Tran-Gyamfi M, Aerts A, Altherr M, Ashworth L, Bajorek E, Black S, Branscomb E, Caenepeel S, Carrano A, Caoile C, Chan YM, Christensen M, Cleland CA, Copeland A, Dalin E, Dehal P, Denys M, Detter JC, Escobar J, Flowers D, Fotopulos D, Garcia C, Georgescu AM, Glavina T, Gomez M, Gonzales E, Groza M, Hammon N, Hawkins T, Haydu L, Ho I, Huang W, Israni S, Jett J, Kadner K, Kimball H, Kobayashi A, Larionov V, Leem SH, Lopez F, Lou Y, Lowry S, Malfatti S, Martinez D, McCready P, Medina C, Morgan J, Nelson K, Nolan M, Ovcharenko I, Pitluck S, Pollard M, Popkie AP, Predki P, Quan G, Ramirez L, Rash S, Retterer J, Rodriguez A, Rogers S, Salamov A, Salazar A, She X, Smith D, Slezak T, Solovyev V, Thayer N, Tice H, Tsai M, Ustaszewska A, Vo N, Wagner M, Wheeler J, Wu K, Xie G, Yang J, Dubchak I, Furey TS, DeJong P, Dickson M, Gordon D, Eichler EE, Pennacchio LA, Richardson P, Stubbs L, Rokhsar DS, Myers RM, Rubin EM, Lucas SM: The DNA sequence and biology of human chromosome 19. Nature. 2004 Apr 1;428(6982):529-35. [PubMed:15057824 ]
  4. Sarto C, Marocchi A, Sanchez JC, Giannone D, Frutiger S, Golaz O, Wilkins MR, Doro G, Cappellano F, Hughes G, Hochstrasser DF, Mocarelli P: Renal cell carcinoma and normal kidney protein expression. Electrophoresis. 1997 Mar-Apr;18(3-4):599-604. [PubMed:9150947 ]
  5. 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. [PubMed:2158323 ]
  6. 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. [PubMed:7721092 ]