Uqcrfs1 ubiquinol-cytochrome c reductase, Rieske iron-sulfur polypeptide 1 [Mus musculus (house mouse)] - Gene

Summary

Official Symbol: Uqcrfs1provided by MGI
Official Full Name: ubiquinol-cytochrome c reductase, Rieske iron-sulfur polypeptide 1provided by MGI
Primary source: MGI:MGI:1913944
See related: Ensembl:ENSMUSG00000038462 AllianceGenome:MGI:1913944
Gene type: protein coding
RefSeq status: PROVISIONAL
Organism: Mus musculus
Lineage: Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha; Muroidea; Muridae; Murinae; Mus; Mus
Also known as: 4430402G14Rik
Summary: Predicted to enable oxidoreductase activity. Predicted to be involved in mitochondrial electron transport, ubiquinol to cytochrome c. Located in mitochondrion. Part of respiratory chain complex III. Is active in mitochondrial inner membrane. Is expressed in several structures, including alimentary system; cardiovascular system; hemolymphoid system; and liver. Used to study mitochondrial metabolism disease. Human ortholog(s) of this gene implicated in mitochondrial complex III deficiency. Orthologous to several human genes including UQCRFS1 (ubiquinol-cytochrome c reductase, Rieske iron-sulfur polypeptide 1). [provided by Alliance of Genome Resources, Dec 2024]
Expression: Ubiquitous expression in adrenal adult (RPKM 429.8), duodenum adult (RPKM 371.7) and 28 other tissues See more
Orthologs: human all
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Genomic context

Location:: 13 A3.2; 13 13.23 cM

Exon count:: 2

Annotation release	Status	Assembly	Chr	Location
RS_2024_02	current	GRCm39 (GCF_000001635.27)	13	NC_000079.7 (30724295..30729299, complement)
108.20200622	previous assembly	GRCm38.p6 (GCF_000001635.26)	13	NC_000079.6 (30540312..30545316, complement)

Chromosome 13 - NC_000079.7 Genomic Context describing neighboring genes

Genomic regions, transcripts, and products

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Genomic Sequence:

Go to nucleotide: Graphics FASTA GenBank

Expression

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See details

Project title: Mouse ENCODE transcriptome data
Description: RNA profiling data sets generated by the Mouse ENCODE project.
BioProject: PRJNA66167
Publication: PMID 25409824
Analysis date: n/a

Bibliography

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GeneRIFs: Gene References Into Functions

What's a GeneRIF?

UQCRFS1 produces N-terminal polypeptides, which remain bound to holocomplex III. We show that, in normal conditions, these UQCRFS1 fragments are rapidly removed, but when TTC19 is absent they accumulate within complex III, causing its structural and functional impairment.
Title: TTC19 Plays a Husbandry Role on UQCRFS1 Turnover in the Biogenesis of Mitochondrial Respiratory Complex III.
Rieske iron-sulfur protein is required for superoxide generation at Complex III.
Title: Superoxide generated at mitochondrial complex III triggers acute responses to hypoxia in the pulmonary circulation.
Mitochondrial respiratory complexes of the electron transport chain (CI and CIV) and supercomplexes were decreased in the RISP-deficient cells.
Title: Cells lacking Rieske iron-sulfur protein have a reactive oxygen species-associated decrease in respiratory complexes I and IV.
A 3' UTR modification of the mitochondrial rieske iron sulfur protein in mice produces a specific skin pigmentation phenotype.
Title: A 3' UTR modification of the mitochondrial rieske iron sulfur protein in mice produces a specific skin pigmentation phenotype.

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Variation

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Alleles

Alleles of this type are documented at Mouse Genome Informatics (MGI)

Endonuclease-mediated (1)
Gene trapped (1)
Targeted (3) 1 citation

Interactions

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Products	Interactant	Other Gene	Complex	Source	Pubs	Description

General gene information

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Markers

AI875505 (e-PCR)
- UniSTS:180510

RH143354 (e-PCR)
- UniSTS:232601

Gene Ontology Provided by MGI

Function	Evidence Code	Pubs
enables 2 iron, 2 sulfur cluster binding	IEA Inferred from Electronic Annotation more info
enables metal ion binding	IEA Inferred from Electronic Annotation more info
enables oxidoreductase activity	IBA Inferred from Biological aspect of Ancestor more info
enables protein binding	IPI Inferred from Physical Interaction more info	PubMed
enables protein-containing complex binding	IEA Inferred from Electronic Annotation more info
enables protein-containing complex binding	ISO Inferred from Sequence Orthology more info
enables ubiquinol-cytochrome-c reductase activity	IEA Inferred from Electronic Annotation more info

Process	Evidence Code	Pubs
involved_in mitochondrial electron transport, ubiquinol to cytochrome c	IBA Inferred from Biological aspect of Ancestor more info
involved_in proton transmembrane transport	IEA Inferred from Electronic Annotation more info
involved_in respiratory electron transport chain	ISS Inferred from Sequence or Structural Similarity more info
involved_in response to hormone	IEA Inferred from Electronic Annotation more info
involved_in response to xenobiotic stimulus	IEA Inferred from Electronic Annotation more info

Component	Evidence Code	Pubs
located_in mitochondrial inner membrane	HDA more info	PubMed
is_active_in mitochondrial inner membrane	IDA Inferred from Direct Assay more info	PubMed
located_in mitochondrial membrane	ISO Inferred from Sequence Orthology more info
located_in mitochondrion	HDA more info	PubMed
located_in mitochondrion	IDA Inferred from Direct Assay more info	PubMed
located_in mitochondrion	ISO Inferred from Sequence Orthology more info
located_in myelin sheath	HDA more info	PubMed
part_of respiratory chain complex III	IBA Inferred from Biological aspect of Ancestor more info
part_of respiratory chain complex III	IDA Inferred from Direct Assay more info	PubMed
part_of respiratory chain complex III	ISO Inferred from Sequence Orthology more info
part_of respiratory chain complex IV	ISO Inferred from Sequence Orthology more info

General protein information

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Preferred Names: cytochrome b-c1 complex subunit Rieske, mitochondrial

Names: RISP; Rieske protein UQCRFS1; complex III subunit 5; cytochrome b-c1 complex subunit 5; rieske iron-sulfur protein; ubiquinol-cytochrome c reductase iron-sulfur subunit

NP_079986.1

EC 7.1.1.8

NCBI Reference Sequences (RefSeq)

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RefSeqs maintained independently of Annotated Genomes

These reference sequences exist independently of genome builds. Explain

These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

mRNA and Protein(s)

NM_025710.2 → NP_079986.1 cytochrome b-c1 complex subunit Rieske, mitochondrial

See identical proteins and their annotated locations for NP_079986.1

Status: PROVISIONAL

Source sequence(s)

AK014470

Consensus CDS

CCDS26416.1

UniProtKB/Swiss-Prot

Q5SVV1, Q9CR68

Related

ENSMUSP00000045284.4, ENSMUST00000042834.4

Conserved Domains (3) summary

cd03470
Location:151 → 274

Rieske_cytochrome_bc1; Iron-sulfur protein (ISP) component of the bc(1) complex family, Rieske domain; The Rieske domain is a [2Fe-2S] cluster binding domain involved in electron transfer. The bc(1) complex is a multisubunit enzyme found in many different organisms including ...

pfam02921
Location:80 → 145

UCR_TM; Ubiquinol cytochrome reductase transmembrane region

pfam09165
Location:2 → 77

Ubiq-Cytc-red_N; Ubiquinol-cytochrome c reductase 8 kDa, N-terminal