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Protein

Cytochrome b-c1 complex subunit Rieske, mitochondrial

Gene

UQCRFS1

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -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 (PubMed:28673544).1 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.

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

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
neXtProtiNX_P47985

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
ProteomicsDBi55828
TopDownProteomicsiP47985

2D gel databases

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

PTM databases

iPTMnetiP47985
PhosphoSitePlusiP47985
SwissPalmiP47985

Expressioni

Gene expression databases

BgeeiENSG00000169021 Expressed in 91 organ(s), highest expression level in muscle of leg
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

Protein-protein interaction databases

BioGridi113232, 162 interactors
ComplexPortaliCPX-560 Mitochondrial respiratory chain complex III
IntActiP47985, 28 interactors
MINTiP47985
STRINGi9606.ENSP00000306397

Structurei

Secondary structure

1274
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

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

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

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...

Protein-protein interaction databases

BioGridi113232, 162 interactors
ComplexPortaliCPX-560 Mitochondrial respiratory chain complex III
IntActiP47985, 28 interactors
MINTiP47985
STRINGi9606.ENSP00000306397

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

PTM databases

iPTMnetiP47985
PhosphoSitePlusiP47985
SwissPalmiP47985

Polymorphism and mutation databases

BioMutaiUQCRFS1
DMDMi143811471

2D gel databases

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

Proteomic databases

EPDiP47985
MaxQBiP47985
PaxDbiP47985
PeptideAtlasiP47985
PRIDEiP47985
ProteomicsDBi55828
TopDownProteomicsiP47985

Protocols and materials databases

DNASUi7386
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000304863; ENSP00000306397; ENSG00000169021
GeneIDi7386
KEGGihsa:7386
UCSCiuc002nsd.3 human

Organism-specific databases

CTDi7386
DisGeNETi7386
EuPathDBiHostDB:ENSG00000169021.5
GeneCardsiUQCRFS1
H-InvDBiHIX0041192
HGNCiHGNC:12587 UQCRFS1
HPAiHPA041863
HPA050339
MIMi191327 gene
neXtProtiNX_P47985
OpenTargetsiENSG00000169021
PharmGKBiPA37218
GenAtlasiSearch...

Phylogenomic databases

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

Enzyme and pathway databases

ReactomeiR-HSA-611105 Respiratory electron transport

Miscellaneous databases

ChiTaRSiUQCRFS1 human
GeneWikiiUQCRFS1
GenomeRNAii7386
PROiPR:P47985
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000169021 Expressed in 91 organ(s), highest expression level in muscle of leg
CleanExiHS_UQCRFS1
GenevisibleiP47985 HS

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
ProtoNetiSearch...

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 7, 2018
This is version 189 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

Keywords - Technical termi

3D-structure, Complete proteome, Direct protein sequencing, Reference proteome

Documents

  1. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  2. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  3. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  4. Human chromosome 19
    Human chromosome 19: entries, gene names and cross-references to MIM
  5. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
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Main funding by: National Institutes of Health

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