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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

Cytochrome b-c1 complex subunit Rieske, mitochondrial: Component of the mitochondrial ubiquinol-cytochrome c reductase complex dimer (complex III dimer), which is a respiratory chain that generates an electrochemical potential coupled to ATP synthesis (PubMed:28673544). Incorporation of UQCRFS1 is the penultimate step in complex III assembly (By similarity).By similarity1 Publication
Cytochrome b-c1 complex subunit 9: Possible component of the mitochondrial ubiquinol-cytochrome c reductase complex dimer (complex III dimer), which is a respiratory chain that generates an electrochemical potential coupled to ATP synthesis (PubMed:28673544). UQCRFS1 undergoes proteolytic processing once it is incorporated in the complex III dimer, including this fragment, called subunit 9, which corresponds to the transit peptide (PubMed:28673544). The proteolytic processing is necessary for the correct insertion of UQCRFS1 in the complex III dimer, but the persistence of UQCRFS1-derived fragments may prevent newly imported UQCRFS1 to be processed and assembled into complex III and is detrimental for the complex III structure and function (PubMed:28673544). It is therefore unsure whether the UQCRFS1 fragments, including this fragment, are structural subunits (PubMed:28673544).1 Publication

Miscellaneous

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

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. Fe-S cluster delivery to the Rieske protein is mediated by components of the iron sulfur (Fe-S) cluster assembly machinery that reside in the mitochondrial matrix (including HSC20 and LYRM7) (PubMed:28380382).PROSITE-ProRule annotation1 Publication

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Metal bindingi217Iron-sulfur (2Fe-2S)PROSITE-ProRule annotation1
Metal bindingi219Iron-sulfur (2Fe-2S); via pros nitrogenPROSITE-ProRule annotation1
Metal bindingi236Iron-sulfur (2Fe-2S)PROSITE-ProRule annotation1
Metal bindingi239Iron-sulfur (2Fe-2S); via pros nitrogenPROSITE-ProRule annotation1

GO - Molecular functioni

GO - Biological processi

Keywordsi

Molecular functionOxidoreductase
Biological processElectron transport, Respiratory chain, Transport
Ligand2Fe-2S, Iron, Iron-sulfur, Metal-binding

Enzyme and pathway databases

ReactomeiR-HSA-611105. 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
Rieske protein UQCRFS1Curated
Ubiquinol-cytochrome c reductase iron-sulfur subunit
Cleaved into the following chain:
Cytochrome b-c1 complex subunit 9By similarity
Short name:
Su9By similarity
Short name:
Subunit 9By similarity
Alternative name(s):
8 kDa subunit 9By similarity
Complex III subunit IX
Cytochrome b-c1 complex subunit 11
Ubiquinol-cytochrome c reductase 8 kDa proteinBy similarity
Gene namesi
Name:UQCRFS1Imported
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 19

Organism-specific databases

EuPathDBiHostDB:ENSG00000169021.5.
HGNCiHGNC:12587. UQCRFS1.
MIMi191327. gene.

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Topology

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transmembranei103 – 140HelicalBy similarityAdd BLAST38

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

Pathology & Biotechi

Organism-specific databases

DisGeNETi7386.
OpenTargetsiENSG00000169021.
PharmGKBiPA37218.

Chemistry databases

DrugBankiDB08453. 2-NONYL-4-HYDROXYQUINOLINE N-OXIDE.
DB07778. FAMOXADONE.
DB07401. METHYL (2Z)-2-(2-{[6-(2-CYANOPHENOXY)PYRIMIDIN-4-YL]OXY}PHENYL)-3-METHOXYACRYLATE.
DB08330. METHYL (2Z)-3-METHOXY-2-{2-[(E)-2-PHENYLVINYL]PHENYL}ACRYLATE.
DB08690. UBIQUINONE-2.

Polymorphism and mutation databases

BioMutaiUQCRFS1.
DMDMi143811471.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 78Mitochondrion1 PublicationAdd BLAST78
ChainiPRO_00003072411 – 78Cytochrome b-c1 complex subunit 91 PublicationAdd BLAST78
ChainiPRO_000003066479 – 274Cytochrome b-c1 complex subunit Rieske, mitochondrialAdd BLAST196

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Disulfide bondi222 ↔ 238PROSITE-ProRule annotation

Post-translational modificationi

Proteolytic processing is necessary for the correct insertion of UQCRFS1 in the complex III dimer (PubMed:28673544). Several fragments are generated during UQCRFS1 insertion, most probably due to the endogenous matrix-processing peptidase (MPP) activity (PubMed:28673544). The action of the protease is also necessary for the clearance of the UQCRFS1 fragments (PubMed:28673544).1 Publication

Keywords - PTMi

Disulfide bond

Proteomic databases

EPDiP47985.
MaxQBiP47985.
PaxDbiP47985.
PeptideAtlasiP47985.
PRIDEiP47985.
TopDownProteomicsiP47985.

2D gel databases

OGPiP47985.
REPRODUCTION-2DPAGEiIPI00026964.
SWISS-2DPAGEiP47985.
UCD-2DPAGEiP47985.

PTM databases

iPTMnetiP47985.
PhosphoSitePlusiP47985.
SwissPalmiP47985.

Expressioni

Gene expression databases

BgeeiENSG00000169021.
CleanExiHS_UQCRFS1.
GenevisibleiP47985. HS.

Organism-specific databases

HPAiHPA041863.
HPA050339.

Interactioni

Subunit structurei

Binds to the mitochondrial respiratory complex III dimer. The monomeric module of the mitochondrial respiratory complex III contains 11 subunits: 3 respiratory subunits involved in its catalytic activity (cytochrome b, cytochrome c1 and Rieske protein 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 subunit 9, the cleavage product of Rieske protein UQCRFS1) (By similarity). Incorporation of the Rieske protein UQCRFS1 is the penultimate step in complex III assembly (PubMed:28380382). Interacts with TTC19, which is involved in the clearance of UQCRFS1 fragments (PubMed:28673544).By similarity2 Publications

Binary interactionsi

WithEntry#Exp.IntActNotes
LYRM7Q5U5X010EBI-1045520,EBI-13943106

GO - Molecular functioni

Protein-protein interaction databases

BioGridi113232. 162 interactors.
IntActiP47985. 20 interactors.
MINTiMINT-2803370.
STRINGi9606.ENSP00000306397.

Structurei

Secondary structure

1274
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details
Feature keyPosition(s)DescriptionActionsGraphical viewLength
Helixi4 – 6Combined sources3
Beta strandi14 – 19Combined sources6
Turni31 – 33Combined sources3
Beta strandi34 – 36Combined sources3
Turni89 – 91Combined sources3
Helixi94 – 96Combined sources3
Beta strandi97 – 101Combined sources5
Helixi104 – 141Combined sources38
Helixi144 – 149Combined sources6
Beta strandi164 – 169Combined sources6
Beta strandi172 – 178Combined sources7
Helixi181 – 189Combined sources9
Turni192 – 194Combined sources3
Beta strandi195 – 197Combined sources3
Helixi201 – 204Combined sources4
Beta strandi210 – 216Combined sources7
Turni218 – 220Combined sources3
Beta strandi225 – 227Combined sources3
Beta strandi232 – 235Combined sources4
Turni237 – 239Combined sources3
Beta strandi242 – 244Combined sources3
Beta strandi249 – 253Combined sources5
Beta strandi263 – 265Combined sources3
Beta strandi270 – 273Combined sources4

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
5XTEelectron microscopy3.40B/O1-57[»]
C/P79-274[»]
5XTHelectron microscopy3.90AB/AO1-57[»]
AC/AP79-274[»]
5XTIelectron microscopy17.40AB/AO1-57[»]
AC/AP79-274[»]
ProteinModelPortaliP47985.
SMRiP47985.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini187 – 272RieskePROSITE-ProRule annotationAdd BLAST86

Keywords - Domaini

Transit peptide, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG1671. Eukaryota.
COG0723. LUCA.
GeneTreeiENSGT00390000001014.
HOVERGENiHBG001040.
InParanoidiP47985.
KOiK00411.
OMAiERKAFSY.
OrthoDBiEOG091G0MOP.
PhylomeDBiP47985.
TreeFamiTF105037.

Family and domain databases

Gene3Di1.20.5.270. 1 hit.
2.102.10.10. 1 hit.
InterProiView protein in InterPro
IPR037008. bc1_Rieske_TM_sf.
IPR011070. Globular_prot_asu/bsu.
IPR017941. Rieske_2Fe-2S.
IPR036922. Rieske_2Fe-2S_sf.
IPR014349. Rieske_Fe-S_prot.
IPR005805. Rieske_Fe-S_prot_C.
IPR004192. Rieske_TM.
IPR015248. Ubiqinol_cyt_c_Rdtase_N.
IPR006317. Ubiquinol_cyt_c_Rdtase_Fe-S-su.
PANTHERiPTHR10134. PTHR10134. 1 hit.
PfamiView protein in Pfam
PF00355. Rieske. 1 hit.
PF09165. Ubiq-Cytc-red_N. 1 hit.
PF02921. UCR_TM. 1 hit.
PRINTSiPR00162. RIESKE.
SUPFAMiSSF50022. SSF50022. 1 hit.
SSF56568. SSF56568. 1 hit.
TIGRFAMsiTIGR01416. Rieske_proteo. 1 hit.
PROSITEiView protein in PROSITE
PS51296. RIESKE. 1 hit.

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)DescriptionActionsGraphical viewLength
Sequence conflicti73P → H in AAH67832 (PubMed:15489334).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_0518636S → A4 PublicationsCorresponds to variant dbSNP:rs8100724Ensembl.1

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.3. human.

Keywords - Coding sequence diversityi

Polymorphism

Similar proteinsi

Entry informationi

Entry nameiUCRI_HUMAN
AccessioniPrimary (citable) accession number: P47985
Secondary accession number(s): A8K519, Q6NVX5, Q9UPH2
Entry historyiIntegrated into UniProtKB/Swiss-Prot: February 1, 1996
Last sequence update: April 3, 2007
Last modified: November 22, 2017
This is version 179 of the entry and version 2 of the sequence. See 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

Caution

Several peptides are generated during UQCRFS1 insertion (PubMed:28673544). According to some authors, the identification of the transit peptide as the subunit 9, does not necessary imply that it must be considered as a structural subunit of the complex III dimer as additional fragments from UQCRFS1 are also present (PubMed:28673544).1 Publication

Keywords - Technical termi

3D-structure, 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. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references