P47985 (UCRI_HUMAN) Reviewed, UniProtKB/Swiss-Prot

Names and origin

Protein names

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:

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

Organism

Homo sapiens (Human) [Reference proteome]

Taxonomic identifier

9606 [NCBI]

Taxonomic lineage

cellular organisms › Eukaryota › Opisthokonta › Metazoa › Eumetazoa › Bilateria › Deuterostomia › Chordata › Craniata › Vertebrata › Gnathostomata › Teleostomi › Euteleostomi › Sarcopterygii › Tetrapoda › Amniota › Mammalia › Theria › Eutheria › Euarchontoglires › Primates › Haplorrhini › Simiiformes › Catarrhini › Hominoidea › Hominidae › Homininae › Homo

Protein attributes

Sequence length	274 AA.
Sequence status	Complete.
Sequence processing	The displayed sequence is further processed into a mature form.
Protein existence	Evidence 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	QH2 + 2 ferricytochrome c = Q + 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

Keywords
Biological process	Electron transport Respiratory chain Transport
Cellular component	Membrane Mitochondrion Mitochondrion inner membrane
Coding sequence diversity	Polymorphism
Domain	Transit peptide Transmembrane Transmembrane helix
Ligand	2Fe-2S Iron Iron-sulfur Metal-binding
Molecular function	Oxidoreductase
PTM	Disulfide bond
Technical term	Complete proteome Direct protein sequencing Reference proteome
Gene Ontology (GO)
Biological_process	respiratory electron transport chain Traceable author statement. Source: Reactome response to antibiotic Inferred from electronic annotation. Source: Compara response to drug Inferred from electronic annotation. Source: Compara response to hormone stimulus Inferred from electronic annotation. Source: Compara small molecule metabolic process Traceable author statement. Source: Reactome
Cellular_component	integral to membrane Inferred from electronic annotation. Source: UniProtKB-KW mitochondrial respiratory chain complex III Non-traceable author statement. Source: UniProtKB
Molecular_function	2 iron, 2 sulfur cluster binding Inferred from electronic annotation. Source: UniProtKB-KW metal ion binding Inferred from electronic annotation. Source: UniProtKB-KW ubiquinol-cytochrome-c reductase activity Non-traceable author statement. Source: UniProtKB
Complete GO annotation...

Sequence annotation (Features)

	Feature key	Position(s)	Length	Description	Graphical view	Feature identifier
Molecule processing
	Chain	1 – 78	78	Cytochrome b-c1 complex subunit 11 By similarity		PRO_0000307241
	Transit peptide	1 – 78	78	Mitochondrion Ref.6
	Chain	79 – 274	196	Cytochrome b-c1 complex subunit Rieske, mitochondrial		PRO_0000030664
Regions
	Transmembrane	103 – 140	38	Helical; By similarity
	Domain	187 – 272	86	Rieske
Sites
	Metal binding	217	1	Iron-sulfur (2Fe-2S) By similarity
	Metal binding	219	1	Iron-sulfur (2Fe-2S); via pros nitrogen By similarity
	Metal binding	236	1	Iron-sulfur (2Fe-2S) By similarity
	Metal binding	239	1	Iron-sulfur (2Fe-2S); via pros nitrogen By similarity
Amino acid modifications
	Disulfide bond	222 ↔ 238		By similarity
Natural variations
	Natural variant	6	1	S → A. Ref.1 Ref.2 Ref.3 Ref.5 Corresponds to variant rs8100724 [ dbSNP | Ensembl ].		VAR_051863
Experimental info
	Sequence conflict	73	1	P → H in AAH67832. Ref.5

Sequences

	Sequence		Length	Mass (Da)
	P47985 [UniParc]. Last modified April 3, 2007. Version 2. Checksum: 8DB51634DEB039B0	FASTA	274	29,668
	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

References

	« Hide 'large scale' referencesShow '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. , 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]	"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.
IPI	IPI00026964.
RefSeq	NP_005994.2. NM_006003.2.
UniGene	Hs.170107.
3D structure databases
ProteinModelPortal	P47985.
ModBase	Search...
Protein-protein interaction databases
IntAct	P47985. 4 interactions.
STRING	9606.ENSP00000306397.
PTM databases
PhosphoSite	P47985.
Polymorphism databases
DMDM	143811471.
2D gel databases
OGP	P47985.
REPRODUCTION-2DPAGE	IPI00026964.
SWISS-2DPAGE	P47985.
UCD-2DPAGE	P47985.
Proteomic databases
PRIDE	P47985.
Protocols and materials databases
DNASU	7386.
StructuralBiologyKnowledgebase	Search...
Genome annotation databases
Ensembl	ENST00000304863; ENSP00000306397; ENSG00000169021.
GeneID	7386.
KEGG	hsa:7386.
UCSC	uc002nsd.2. human.
Organism-specific databases
CTD	7386.
GeneCards	GC19M029698.
H-InvDB	HIX0041192.
HGNC	HGNC:12587. UQCRFS1.
HPA	HPA041863.
MIM	191327. gene.
neXtProt	NX_P47985.
PharmGKB	PA37218.
GenAtlas	Search...
Phylogenomic databases
HOVERGEN	HBG001040.
InParanoid	P47985.
KO	K00411.
OMA	DYRRAEV.
PhylomeDB	P47985.
Enzyme and pathway databases
Reactome	REACT_111217. Metabolism.
Gene expression databases
Bgee	P47985.
CleanEx	HS_UQCRFS1.
Genevestigator	P47985.
GermOnline	ENSG00000169021. Homo sapiens.
Family and domain databases
Gene3D	1.20.5.270. 1 hit. 2.102.10.10. 1 hit.
InterPro	IPR011070. 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]
PANTHER	PTHR10134. PTHR10134. 1 hit.
Pfam	PF00355. Rieske. 1 hit. PF09165. Ubiq-Cytc-red_N. 1 hit. PF02921. UCR_TM. 1 hit. [Graphical view]
PRINTS	PR00162. RIESKE.
SUPFAM	SSF56568. AlphaBeta_subunt. 1 hit. SSF50022. Rieske_dom. 1 hit.
TIGRFAMs	TIGR01416. Rieske_proteo. 1 hit.
PROSITE	PS51296. RIESKE. 1 hit. [Graphical view]
ProtoNet	Search...
Other
ChiTaRS	UQCRFS1. human.
GenomeRNAi	7386.
NextBio	28920.
SOURCE	Search...

Entry information

Entry name

UCRI_HUMAN

Accession

Primary (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:	May 1, 2013
This is version 136 of the entry and version 2 of the sequence. [Complete history]

Entry status

Reviewed (UniProtKB/Swiss-Prot)

Annotation program

Chordata Protein Annotation Program

Disclaimer

Any 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.