UQCRFS1

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Ubiquinol-cytochrome c reductase, Rieske iron-sulfur polypeptide 1

PDB rendering based on 1be3.

Available structures

Ortholog search: PDBe, RCSB

List of PDB id codes
1be3, 1bgy, 1l0l, 1l0n, 1ntk, 1ntm, 1ntz, 1nu1, 1pp9, 1ppj, 1qcr, 1rie, 1sqb, 1sqp, 1sqq, 1sqv, 1sqx, 2a06, 2fyu, 2ibz, 2qjk, 2qjp, 2qjy, 3cwb, 3cx5, 3cxh, 3h1h, 3h1i, 3h1j, 3h1k, 3h1l, 3l70, 3l71, 3l72, 3l73, 3l74, 3l75

Identifiers

UQCRFS1; RIP1; RIS1; RISP; UQCR5

External IDs

OMIM: 191327 MGI: 1913944 HomoloGene: 4378 GeneCards: UQCRFS1 Gene

Gene Ontology
Molecular function	• ubiquinol-cytochrome-c reductase activity • protein complex binding • metal ion binding • 2 iron, 2 sulfur cluster binding
Cellular component	• mitochondrion • mitochondrial inner membrane • mitochondrial respiratory chain complex III • integral to membrane
Biological process	• response to hormone stimulus • respiratory electron transport chain • response to drug • small molecule metabolic process • response to antibiotic • oxidation-reduction process
Sources: Amigo / QuickGO

RNA expression pattern

More reference expression data

Orthologs

Species

Human

Mouse

ENSG00000169021

ENSMUSG00000038462

RefSeq (mRNA)

RefSeq (protein)

Location (UCSC)

Chr 19:
29.7 – 29.7 Mb

Chr 13:
30.54 – 30.55 Mb

PubMed search

Ubiquinol-cytochrome c reductase, Rieske iron-sulfur polypeptide 1, also known as UQCRFS1, is a protein which in humans is encoded by the UQCRFS1 gene.^[1]

UQCRFS1 is a subunit of the respiratory chain protein Ubiquinol Cytochrome c Reductase (UQCR, Complex III or Cytochrome bc1 complex), which consists of the products of one mitochondrially encoded gene:

MTCYTB (mitochondrial cytochrome b)

and ten nuclear genes:

UQCRC1, UQCRC2, Cytochrome C1, UQCRFS1 (this protein, a type of Rieske protein), UQCRB,UQCRQ ("11kDa protein"), UQCRH (cyt c1 Hinge protein), UCRC ("cyt. c1 associated protein"), and UQCR ("Rieske-associated protein").

After processing the cleaved leader sequence of the iron-sulfur protein is retained as subunit 9, giving 11 subunits from 10 genes.

References

↑ "Entrez Gene: UQCRFS1 ubiquinol-cytochrome c reductase, Rieske iron-sulfur polypeptide 1".

Further reading

Nishikimi M, Hosokawa Y, Toda H, et al. (1990). "The primary structure of human Rieske iron-sulfur protein of mitochondrial cytochrome bc1 complex deduced from cDNA analysis.". Biochem. Int. 20 (1): 155–60. PMID 2158323.
Shimomura Y, Nishikimi M, Ozawa T (1986). "Novel purification of cytochrome c1 from mitochondrial Complex III. Reconstitution of antimycin-insensitive electron transfer with the iron-sulfur protein and cytochrome c1.". J. Biol. Chem. 260 (28): 15075–80. PMID 2999105.
Pennacchio LA, Bergmann A, Fukushima A, et al. (1995). "Structure, sequence and location of the UQCRFS1 gene for the human Rieske Fe-S protein.". Gene 155 (2): 207–11. doi:10.1016/0378-1119(94)00683-J. PMID 7721092.
Duncan AM, Anderson L, Duff C, et al. (1994). "Assignment of the gene (UQCRFS1) for the Rieske iron-sulfur protein subunit of the mitochondrial cytochrome bc1 complex to the 22q13 and 19q12-q13.1 regions of the human genome.". Genomics 21 (1): 281–3. doi:10.1006/geno.1994.1260. PMID 8088805.
Sarto C, Marocchi A, Sanchez JC, et al. (1997). "Renal cell carcinoma and normal kidney protein expression.". Electrophoresis 18 (3–4): 599–604. doi:10.1002/elps.1150180343. PMID 9150947.
Strausberg RL, Feingold EA, Grouse LH, et al. (2003). "Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences". Proc. Natl. Acad. Sci. U.S.A. 99 (26): 16899–903. doi:10.1073/pnas.242603899. PMC 139241. PMID 12477932.
Kaneko SJ, Gerasimova T, Smith ST, et al. (2003). "CA125 and UQCRFS1 FISH studies of ovarian carcinoma". Gynecol. Oncol. 90 (1): 29–36. doi:10.1016/S0090-8258(03)00144-6. PMID 12821338.
Ohashi Y, Kaneko SJ, Cupples TE, Young SR (2004). "Ubiquinol cytochrome c reductase (UQCRFS1) gene amplification in primary breast cancer core biopsy samples". Gynecol. Oncol. 93 (1): 54–8. doi:10.1016/j.ygyno.2004.01.019. PMID 15047214.
Grimwood J, Gordon LA, Olsen A, et al. (2004). "The DNA sequence and biology of human chromosome 19". Nature 428 (6982): 529–35. doi:10.1038/nature02399. PMID 15057824.
Gerhard DS, Wagner L, Feingold EA, et al. (2004). "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)". Genome Res. 14 (10B): 2121–7. doi:10.1101/gr.2596504. PMC 528928. PMID 15489334.

PDB gallery

1be3: CYTOCHROME BC1 COMPLEX FROM BOVINE

1bgy: CYTOCHROME BC1 COMPLEX FROM BOVINE

1l0l: structure of bovine mitochondrial cytochrome bc1 complex with a bound fungicide famoxadone

1l0n: native structure of bovine mitochondrial cytochrome bc1 complex

1ntk: Crystal Structure of Mitochondrial Cytochrome bc1 in Complex with Antimycin A1

1ntm: Crystal Structure of Mitochondrial Cytochrome bc1 Complex at 2.4 Angstrom

1ntz: Crystal Structure of Mitochondrial Cytochrome bc1 Complex Bound with Ubiquinone

1nu1: Crystal Structure of Mitochondrial Cytochrome bc1 Complexed with 2-nonyl-4-hydroxyquinoline N-oxide (NQNO)

1pp9: Bovine cytochrome bc1 complex with stigmatellin bound

1ppj: Bovine cytochrome bc1 complex with stigmatellin and antimycin

1qcr: CRYSTAL STRUCTURE OF BOVINE MITOCHONDRIAL CYTOCHROME BC1 COMPLEX, ALPHA CARBON ATOMS ONLY

1rie: STRUCTURE OF A WATER SOLUBLE FRAGMENT OF THE RIESKE IRON-SULFUR PROTEIN OF THE BOVINE HEART MITOCHONDRIAL CYTOCHROME BC1-COMPLEX

1sqb: Crystal Structure Analysis of Bovine Bc1 with Azoxystrobin

1sqp: Crystal Structure Analysis of Bovine Bc1 with Myxothiazol

1sqq: Crystal Structure Analysis of Bovine Bc1 with Methoxy Acrylate Stilbene (MOAS)

1sqv: Crystal Structure Analysis of Bovine Bc1 with UHDBT

1sqx: Crystal Structure Analysis of Bovine Bc1 with Stigmatellin A

2a06: Bovine cytochrome bc1 complex with stigmatellin bound

2fyu: Crystal structure of bovine heart mitochondrial bc1 with jg144 inhibitor

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