Reviewed, UniProtKB/Swiss-Prot P47985 (UCRI_HUMAN)

Names and origin

Protein names

Recommended name:
    Cytochrome b-c1 complex subunit Rieske, mitochondrial
    EC=1.10.2.2
Alternative name(s):
    Ubiquinol-cytochrome c reductase iron-sulfur subunit
    Rieske iron-sulfur protein
      Short name=RISP
    Complex III subunit 5
Cleaved into the following chain:
    1- Recommended name:
            Cytochrome b-c1 complex subunit 11
        Alternative name(s):
            Ubiquinol-cytochrome c reductase 8 kDa protein
            Complex III subunit IX

Gene names

Name:

UQCRFS1

Organism

Homo sapiens (Human)

Taxonomic identifier

9606 [NCBI]

Taxonomic lineage

Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › 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.
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
Ligand	2Fe-2S Iron Iron-sulfur Metal-binding
Molecular function	Oxidoreductase
PTM	Disulfide bond
Technical term	Direct protein sequencing
Gene Ontology (GO)
Biological process	electron transport chain Inferred from electronic annotation. Source: UniProtKB-KW transport Inferred from electronic annotation. Source: UniProtKB-KW
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 electron carrier activity Inferred from electronic annotation. Source: InterPro iron 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
	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: dbSNP rs8100724. Ref.1 Ref.2 Ref.3 Ref.5		VAR_051863
Experimental info
	Sequence conflict	73	1	P → H in AAH67832. Ref.5

Sequences

	Sequence		Length	Mass (Da)
	P47985-1 [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: 2158323] [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: 7721092] [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: 14702039] [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: 15057824] [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: 15489334] [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: 9150947] [Abstract] Cited for: PARTIAL PROTEIN SEQUENCE OF 79-90. Tissue: Kidney.
[7]	Colinge J., Superti-Furga G., Bennett K.L. Submitted (OCT-2008) to UniProtKB Cited for: IDENTIFICATION [LARGE SCALE ANALYSIS], MASS SPECTROMETRY.
+	Additional computationally mapped references.

Cross-references

Sequence databases
EMBLGenBankDDBJ	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.
UniGene	Hs.170107
3D structure databases
HSSP	HSSP built from PDB template 1RIE based on UniProtKB P13272.
SMR	P47985. Positions 1-57, 79-274.
ModBase	Search...
Protein-protein interaction databases
IntAct	P47985. 2 interactions.
PTM databases
PhosphoSite	P47985.
2-D gel databases
SWISS-2DPAGE	P47985.
HSC-2DPAGE	P47985.
OGP	P47985.
REPRODUCTION-2DPAGE	IPI00026964.
Proteomic databases
PRIDE	P47985.
Genome annotation databases
Ensembl	ENSG00000169021. Homo sapiens. [Contig view]
GeneID	7386.
KEGG	hsa:7386.
NMPDR	fig|9606.3.peg.16154.
Organism-specific databases
GeneCards	GC19M034390. GC22P038593.
H-InvDB	HIX0014973. HIX0041192. HIX0080950.
HGNC	HGNC:12587. UQCRFS1.
MIM	191327. gene.
PharmGKB	PA37218.
GenAtlas	Search...
Phylogenomic databases
HOGENOM	P47985.
HOVERGEN	P47985.
OMA	P47985. DYRRAEV.
Enzyme and pathway databases
BRENDA	1.10.2.2. 247.
Reactome	REACT_6305. Electron Transport Chain.
Gene expression databases
ArrayExpress	P47985.
CleanEx	HS_UQCRFS1.
GermOnline	ENSG00000169021. Homo sapiens.
Family and domain databases
InterPro	IPR017941. Rieske_2Fe-2S. IPR014349. Rieske_Fe-S_prot. IPR015248. Ubiqinol_cyt_c_Rdtase_N. IPR006317. Ubiquinol_cyt_c_Rdtase_Fe-S-su. IPR004192. Ubiquinol_cyt_Rdtase_TM. [Graphical view]
Gene3D	G3DSA:2.102.10.10. Rieske_reg. 1 hit.
PANTHER	PTHR10134. Rieske. 1 hit.
Pfam	PF00355. Rieske. 1 hit. PF09165. Ubiq-Cytc-red_N. 1 hit. PF02921. UCR_TM. 1 hit. [Graphical view]
TIGRFAMs	TIGR01416. Rieske_proteo. 1 hit.
PROSITE	PS51296. RIESKE. 1 hit. [Graphical view]
ProtoNet	Search...
Other Resources
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:	June 16, 2009
This is version 94 of the entry and version 2 of the sequence. [Complete history]

Entry status

Reviewed (UniProtKB/Swiss-Prot)

Annotation project

HPI (Human Proteome Initiative)