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Protein

Cytochrome b-c1 complex subunit Rieske, mitochondrial

Gene

UQCRFS1

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

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 activityi

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

Cofactori

[2Fe-2S] clusterPROSITE-ProRule annotationNote: Binds 1 [2Fe-2S] cluster per subunit.PROSITE-ProRule annotation

Sites

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Metal bindingi217 – 2171Iron-sulfur (2Fe-2S)PROSITE-ProRule annotation
Metal bindingi219 – 2191Iron-sulfur (2Fe-2S); via pros nitrogenPROSITE-ProRule annotation
Metal bindingi236 – 2361Iron-sulfur (2Fe-2S)PROSITE-ProRule annotation
Metal bindingi239 – 2391Iron-sulfur (2Fe-2S); via pros nitrogenPROSITE-ProRule annotation

GO - Molecular functioni

GO - Biological processi

Complete GO annotation...

Keywords - Molecular functioni

Oxidoreductase

Keywords - Biological processi

Electron transport, Respiratory chain, Transport

Keywords - Ligandi

2Fe-2S, Iron, Iron-sulfur, Metal-binding

Enzyme and pathway databases

ReactomeiREACT_22393. Respiratory electron transport.

Names & Taxonomyi

Protein namesi
Recommended 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:
Alternative name(s):
Complex III subunit IX
Ubiquinol-cytochrome c reductase 8 kDa protein
Gene namesi
Name:UQCRFS1
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
ProteomesiUP000005640 Componenti: Chromosome 19

Organism-specific databases

HGNCiHGNC:12587. UQCRFS1.

Subcellular locationi

Topology

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Transmembranei103 – 14038HelicalBy similarityAdd
BLAST

GO - Cellular componenti

Complete GO annotation...

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

Pathology & Biotechi

Organism-specific databases

PharmGKBiPA37218.

Polymorphism and mutation databases

BioMutaiUQCRFS1.
DMDMi143811471.

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Transit peptidei1 – 7878Mitochondrion1 PublicationAdd
BLAST
Chaini1 – 7878Cytochrome b-c1 complex subunit 11By similarityPRO_0000307241Add
BLAST
Chaini79 – 274196Cytochrome b-c1 complex subunit Rieske, mitochondrialPRO_0000030664Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Disulfide bondi222 ↔ 238PROSITE-ProRule annotation

Keywords - PTMi

Disulfide bond

Proteomic databases

MaxQBiP47985.
PRIDEiP47985.

2D gel databases

OGPiP47985.
REPRODUCTION-2DPAGEIPI00026964.
SWISS-2DPAGEP47985.
UCD-2DPAGEP47985.

PTM databases

PhosphoSiteiP47985.

Expressioni

Gene expression databases

BgeeiP47985.
CleanExiHS_UQCRFS1.
GenevisibleiP47985. HS.

Organism-specific databases

HPAiHPA041863.
HPA050339.

Interactioni

Subunit structurei

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).

Protein-protein interaction databases

BioGridi113232. 36 interactions.
IntActiP47985. 11 interactions.
MINTiMINT-2803370.
STRINGi9606.ENSP00000306397.

Structurei

3D structure databases

ProteinModelPortaliP47985.
SMRiP47985. Positions 1-57, 79-274.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Domaini187 – 27286RieskePROSITE-ProRule annotationAdd
BLAST

Sequence similaritiesi

Contains 1 Rieske domain.PROSITE-ProRule annotation

Keywords - Domaini

Transit peptide, Transmembrane, Transmembrane helix

Phylogenomic databases

GeneTreeiENSGT00390000001014.
HOVERGENiHBG001040.
InParanoidiP47985.
KOiK00411.
OMAiPEWIILI.
OrthoDBiEOG7GBFXQ.
PhylomeDBiP47985.
TreeFamiTF105037.

Family and domain databases

Gene3Di1.20.5.270. 1 hit.
2.102.10.10. 1 hit.
InterProiIPR011070. 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]
PANTHERiPTHR10134. PTHR10134. 1 hit.
PfamiPF00355. Rieske. 1 hit.
PF09165. Ubiq-Cytc-red_N. 1 hit.
PF02921. UCR_TM. 1 hit.
[Graphical view]
PRINTSiPR00162. RIESKE.
SUPFAMiSSF50022. SSF50022. 1 hit.
SSF56568. SSF56568. 1 hit.
TIGRFAMsiTIGR01416. Rieske_proteo. 1 hit.
PROSITEiPS51296. RIESKE. 1 hit.
[Graphical view]

Sequencei

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

P47985-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MLSVASRSGP FAPVLSATSR GVAGALRPLV QATVPATPEQ PVLDLKRPFL
60 70 80 90 100
SRESLSGQAV RRPLVASVGL NVPASVCYSH TDIKVPDFSE YRRLEVLDST
110 120 130 140 150
KSSRESSEAR KGFSYLVTGV TTVGVAYAAK NAVTQFVSSM SASADVLALA
160 170 180 190 200
KIEIKLSDIP EGKNMAFKWR GKPLFVRHRT QKEIEQEAAV ELSQLRDPQH
210 220 230 240 250
DLDRVKKPEW VILIGVCTHL GCVPIANAGD FGGYYCPCHG SHYDASGRIR
260 270
LGPAPLNLEV PTYEFTSDDM VIVG
Length:274
Mass (Da):29,668
Last modified:April 3, 2007 - v2
Checksum:i8DB51634DEB039B0
GO

Experimental Info

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Sequence conflicti73 – 731P → H in AAH67832 (PubMed:15489334).Curated

Natural variant

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti6 – 61S → A.4 Publications
Corresponds to variant rs8100724 [ dbSNP | Ensembl ].
VAR_051863

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
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.
CCDSiCCDS12415.1.
RefSeqiNP_005994.2. NM_006003.2.
UniGeneiHs.743307.

Genome annotation databases

EnsembliENST00000304863; ENSP00000306397; ENSG00000169021.
GeneIDi7386.
KEGGihsa:7386.
UCSCiuc002nsd.2. human.

Keywords - Coding sequence diversityi

Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
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.
CCDSiCCDS12415.1.
RefSeqiNP_005994.2. NM_006003.2.
UniGeneiHs.743307.

3D structure databases

ProteinModelPortaliP47985.
SMRiP47985. Positions 1-57, 79-274.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi113232. 36 interactions.
IntActiP47985. 11 interactions.
MINTiMINT-2803370.
STRINGi9606.ENSP00000306397.

PTM databases

PhosphoSiteiP47985.

Polymorphism and mutation databases

BioMutaiUQCRFS1.
DMDMi143811471.

2D gel databases

OGPiP47985.
REPRODUCTION-2DPAGEIPI00026964.
SWISS-2DPAGEP47985.
UCD-2DPAGEP47985.

Proteomic databases

MaxQBiP47985.
PRIDEiP47985.

Protocols and materials databases

DNASUi7386.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000304863; ENSP00000306397; ENSG00000169021.
GeneIDi7386.
KEGGihsa:7386.
UCSCiuc002nsd.2. human.

Organism-specific databases

CTDi7386.
GeneCardsiGC19M029698.
H-InvDBHIX0041192.
HGNCiHGNC:12587. UQCRFS1.
HPAiHPA041863.
HPA050339.
MIMi191327. gene.
neXtProtiNX_P47985.
PharmGKBiPA37218.
GenAtlasiSearch...

Phylogenomic databases

GeneTreeiENSGT00390000001014.
HOVERGENiHBG001040.
InParanoidiP47985.
KOiK00411.
OMAiPEWIILI.
OrthoDBiEOG7GBFXQ.
PhylomeDBiP47985.
TreeFamiTF105037.

Enzyme and pathway databases

ReactomeiREACT_22393. Respiratory electron transport.

Miscellaneous databases

ChiTaRSiUQCRFS1. human.
GeneWikiiUQCRFS1.
GenomeRNAii7386.
NextBioi28920.
PROiP47985.
SOURCEiSearch...

Gene expression databases

BgeeiP47985.
CleanExiHS_UQCRFS1.
GenevisibleiP47985. HS.

Family and domain databases

Gene3Di1.20.5.270. 1 hit.
2.102.10.10. 1 hit.
InterProiIPR011070. 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]
PANTHERiPTHR10134. PTHR10134. 1 hit.
PfamiPF00355. Rieske. 1 hit.
PF09165. Ubiq-Cytc-red_N. 1 hit.
PF02921. UCR_TM. 1 hit.
[Graphical view]
PRINTSiPR00162. RIESKE.
SUPFAMiSSF50022. SSF50022. 1 hit.
SSF56568. SSF56568. 1 hit.
TIGRFAMsiTIGR01416. Rieske_proteo. 1 hit.
PROSITEiPS51296. RIESKE. 1 hit.
[Graphical view]
ProtoNetiSearch...

Publicationsi

« Hide 'large scale' publications
  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.
    , 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.
    , 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. Cited for: PROTEIN SEQUENCE OF 79-90.
    Tissue: Kidney.
  7. Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].

Entry informationi

Entry nameiUCRI_HUMAN
AccessioniPrimary (citable) accession number: P47985
Secondary accession number(s): A8K519, Q6NVX5, Q9UPH2
Entry historyi
Integrated into UniProtKB/Swiss-Prot: February 1, 1996
Last sequence update: April 3, 2007
Last modified: July 22, 2015
This is version 157 of the entry and version 2 of the sequence. [Complete history]
Entry statusiReviewed (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.

Miscellaneousi

Miscellaneous

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

Keywords - Technical termi

Complete proteome, Direct protein sequencing, Reference proteome

Documents

  1. Human chromosome 19
    Human chromosome 19: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. SIMILARITY comments
    Index of protein domains and families

External Data

Dasty 3

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into Uniref entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.