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P47985 (UCRI_HUMAN) Reviewed, UniProtKB/Swiss-Prot

Last modified July 9, 2014. Version 146. Feed History...

Clusters with 100%, 90%, 50% identity | Documents (5) | Third-party data text xml rdf/xml gff fasta
to top of pageNames·Attributes·General annotation·Ontologies·Sequence annotation·Sequences·References·Cross-refs·Entry info·DocumentsCustomize order

Names and origin

Protein namesRecommended name:
Cytochrome b-c1 complex subunit Rieske, mitochondrial

EC=1.10.2.2
Alternative name(s):
Complex III subunit 5
Cytochrome b-c1 complex subunit 5
Rieske iron-sulfur protein
Short name=RISP
Ubiquinol-cytochrome c reductase iron-sulfur subunit

Cleaved into the following chain:

  1. Cytochrome b-c1 complex subunit 11
    Alternative name(s):
    Complex III subunit IX
    Ubiquinol-cytochrome c reductase 8 kDa protein
Gene names
Name:UQCRFS1
OrganismHomo sapiens (Human) [Reference proteome]
Taxonomic identifier9606 [NCBI]
Taxonomic lineageEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo

Protein attributes

Sequence length274 AA.
Sequence statusComplete.
Sequence processingThe displayed sequence is further processed into a mature form.
Protein existenceEvidence at protein level

General annotation (Comments)

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.

Catalytic activity

Quinol + 2 ferricytochrome c = quinone + 2 ferrocytochrome c + 2 H+.

Cofactor

Binds 1 2Fe-2S cluster per subunit By similarity.

Subunit structure

The bc1 complex contains 11 subunits: 3 respiratory subunits (cytochrome b, cytochrome c1 and Rieske/UQCRFS1), 2 core proteins (UQCRC1/QCR1 and UQCRC2/QCR2) and 6 low-molecular weight proteins (UQCRH/QCR6, UQCRB/QCR7, UQCRQ/QCR8, UQCR10/QCR9, UQCR11/QCR10 and a cleavage product of Rieske/UQCRFS1).

Subcellular location

Mitochondrion inner membrane; Single-pass membrane protein.

Miscellaneous

The Rieske protein is a high potential 2Fe-2S protein.

Sequence similarities

Contains 1 Rieske domain.

Ontologies

Sequence annotation (Features)

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifier

Molecule processing

Chain1 – 7878Cytochrome b-c1 complex subunit 11 By similarity
PRO_0000307241
Transit peptide1 – 7878Mitochondrion Ref.6
Chain79 – 274196Cytochrome b-c1 complex subunit Rieske, mitochondrial
PRO_0000030664

Regions

Transmembrane103 – 14038Helical; By similarity
Domain187 – 27286Rieske

Sites

Metal binding2171Iron-sulfur (2Fe-2S) By similarity
Metal binding2191Iron-sulfur (2Fe-2S); via pros nitrogen By similarity
Metal binding2361Iron-sulfur (2Fe-2S) By similarity
Metal binding2391Iron-sulfur (2Fe-2S); via pros nitrogen By similarity

Amino acid modifications

Disulfide bond222 ↔ 238 By similarity

Natural variations

Natural variant61S → A. Ref.1 Ref.2 Ref.3 Ref.5
Corresponds to variant rs8100724 [ dbSNP | Ensembl ].
VAR_051863

Experimental info

Sequence conflict731P → H in AAH67832. Ref.5

Sequences

Sequence LengthMass (Da)Tools
P47985 [UniParc].

Last modified April 3, 2007. Version 2.
Checksum: 8DB51634DEB039B0

FASTA27429,668
        10         20         30         40         50         60 
MLSVASRSGP FAPVLSATSR GVAGALRPLV QATVPATPEQ PVLDLKRPFL SRESLSGQAV 

        70         80         90        100        110        120 
RRPLVASVGL NVPASVCYSH TDIKVPDFSE YRRLEVLDST KSSRESSEAR KGFSYLVTGV 

       130        140        150        160        170        180 
TTVGVAYAAK NAVTQFVSSM SASADVLALA KIEIKLSDIP EGKNMAFKWR GKPLFVRHRT 

       190        200        210        220        230        240 
QKEIEQEAAV ELSQLRDPQH DLDRVKKPEW VILIGVCTHL GCVPIANAGD FGGYYCPCHG 

       250        260        270 
SHYDASGRIR LGPAPLNLEV PTYEFTSDDM VIVG 

« Hide

References

« Hide 'large scale' references
[1]"The primary structure of human Rieske iron-sulfur protein of mitochondrial cytochrome bc1 complex deduced from cDNA analysis."
Nishikimi M., Hosokawa Y., Toda H., Suzuki H., Ozawa T.
Biochem. Int. 20:155-160(1990) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [MRNA], VARIANT ALA-6.
[2]"Structure, sequence and location of the UQCRFS1 gene for the human Rieske Fe-S protein."
Pennacchio L., Bergmann A., Fukushima A., Salemi A., Okubo K., Lennon G.
Gene 155:207-211(1995) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA], VARIANT ALA-6.
[3]"Complete sequencing and characterization of 21,243 full-length human cDNAs."
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. expand/collapse author list , 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 T.-O., 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.
Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA], VARIANT ALA-6.
[4]"The DNA sequence and biology of human chromosome 19."
Grimwood J., Gordon L.A., Olsen A.S., Terry A., Schmutz J., Lamerdin J.E., 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.V. expand/collapse author list , Caoile C., Chan Y.M., Christensen M., Cleland C.A., Copeland A., Dalin E., Dehal P., Denys M., Detter J.C., Escobar J., Flowers D., Fotopulos D., Garcia C., Georgescu A.M., 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 S.-H., Lopez F., Lou Y., Lowry S., Malfatti S., Martinez D., McCready P.M., Medina C., Morgan J., Nelson K., Nolan M., Ovcharenko I., Pitluck S., Pollard M., Popkie A.P., 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 T.S., DeJong P., Dickson M., Gordon D., Eichler E.E., Pennacchio L.A., Richardson P., Stubbs L., Rokhsar D.S., Myers R.M., Rubin E.M., Lucas S.M.
Nature 428:529-535(2004) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
[5]"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
The MGC Project Team
Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA], VARIANT ALA-6.
Tissue: Brain, Lung and Lymph.
[6]"Renal cell carcinoma and normal kidney protein expression."
Sarto C., Marocchi A., Sanchez J.-C., Giannone B., Frutiger S., Golaz O., Wilkins M.R., Doro G., Cappellano F., Hughes G.J., Hochstrasser D.F., Mocarelli P.
Electrophoresis 18:599-604(1997) [PubMed] [Europe PMC] [Abstract]
Cited for: PROTEIN SEQUENCE OF 79-90.
Tissue: Kidney.
[7]"Initial characterization of the human central proteome."
Burkard T.R., Planyavsky M., Kaupe I., Breitwieser F.P., Buerckstuemmer T., Bennett K.L., Superti-Furga G., Colinge J.
BMC Syst. Biol. 5:17-17(2011) [PubMed] [Europe PMC] [Abstract]
Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
+Additional computationally mapped references.

Cross-references

Sequence databases

EMBL
GenBank
DDBJ
L32977, L32917 Genomic DNA. Translation: AAC41754.1.
AK291134 mRNA. Translation: BAF83823.1.
AC007786 Genomic DNA. Translation: AAD38242.1.
BC000649 mRNA. Translation: AAH00649.1.
BC010035 mRNA. Translation: AAH10035.1.
BC067832 mRNA. Translation: AAH67832.1.
CCDSCCDS12415.1.
RefSeqNP_005994.2. NM_006003.2.
UniGeneHs.743307.

3D structure databases

ProteinModelPortalP47985.
SMRP47985. Positions 1-57, 79-274.
ModBaseSearch...
MobiDBSearch...

Protein-protein interaction databases

BioGrid113232. 33 interactions.
IntActP47985. 6 interactions.
MINTMINT-2803370.
STRING9606.ENSP00000306397.

PTM databases

PhosphoSiteP47985.

Polymorphism databases

DMDM143811471.

2D gel databases

OGPP47985.
REPRODUCTION-2DPAGEIPI00026964.
SWISS-2DPAGEP47985.
UCD-2DPAGEP47985.

Proteomic databases

MaxQBP47985.
PRIDEP47985.

Protocols and materials databases

DNASU7386.
StructuralBiologyKnowledgebaseSearch...

Genome annotation databases

EnsemblENST00000304863; ENSP00000306397; ENSG00000169021.
GeneID7386.
KEGGhsa:7386.
UCSCuc002nsd.2. human.

Organism-specific databases

CTD7386.
GeneCardsGC19M029698.
H-InvDBHIX0041192.
HGNCHGNC:12587. UQCRFS1.
HPAHPA041863.
MIM191327. gene.
neXtProtNX_P47985.
PharmGKBPA37218.
GenAtlasSearch...

Phylogenomic databases

HOVERGENHBG001040.
InParanoidP47985.
KOK00411.
OMADYRRAEV.
OrthoDBEOG7GBFXQ.
PhylomeDBP47985.
TreeFamTF105037.

Enzyme and pathway databases

ReactomeREACT_111217. Metabolism.

Gene expression databases

BgeeP47985.
CleanExHS_UQCRFS1.
GenevestigatorP47985.

Family and domain databases

Gene3D1.20.5.270. 1 hit.
2.102.10.10. 1 hit.
InterProIPR011070. Globular_prot_asu/bsu.
IPR017941. Rieske_2Fe-2S.
IPR014349. Rieske_Fe-S_prot.
IPR005805. Rieske_Fe-S_prot_C.
IPR015248. Ubiqinol_cyt_c_Rdtase_N.
IPR006317. Ubiquinol_cyt_c_Rdtase_Fe-S-su.
IPR004192. Ubiquinol_cyt_Rdtase_TM.
[Graphical view]
PANTHERPTHR10134. PTHR10134. 1 hit.
PfamPF00355. Rieske. 1 hit.
PF09165. Ubiq-Cytc-red_N. 1 hit.
PF02921. UCR_TM. 1 hit.
[Graphical view]
PRINTSPR00162. RIESKE.
SUPFAMSSF50022. SSF50022. 1 hit.
SSF56568. SSF56568. 1 hit.
TIGRFAMsTIGR01416. Rieske_proteo. 1 hit.
PROSITEPS51296. RIESKE. 1 hit.
[Graphical view]
ProtoNetSearch...

Other

ChiTaRSUQCRFS1. human.
GeneWikiUQCRFS1.
GenomeRNAi7386.
NextBio28920.
PROP47985.
SOURCESearch...

Entry information

Entry nameUCRI_HUMAN
AccessionPrimary (citable) accession number: P47985
Secondary accession number(s): A8K519, Q6NVX5, Q9UPH2
Entry history
Integrated into UniProtKB/Swiss-Prot: February 1, 1996
Last sequence update: April 3, 2007
Last modified: July 9, 2014
This is version 146 of the entry and version 2 of the sequence. [Complete history]
Entry statusReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Relevant documents

SIMILARITY comments

Index of protein domains and families

MIM cross-references

Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot

Human polymorphisms and disease mutations

Index of human polymorphisms and disease mutations

Human entries with polymorphisms or disease mutations

List of human entries with polymorphisms or disease mutations

Human chromosome 19

Human chromosome 19: entries, gene names and cross-references to MIM