Uqcrh ubiquinol-cytochrome c reductase hinge protein [Mus musculus (house mouse)] - Gene

Summary

Official Symbol: Uqcrhprovided by MGI
Official Full Name: ubiquinol-cytochrome c reductase hinge proteinprovided by MGI
Primary source: MGI:MGI:1913826
See related: Ensembl:ENSMUSG00000063882 AllianceGenome:MGI:1913826
Gene type: protein coding
RefSeq status: VALIDATED
Organism: Mus musculus
Lineage: Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha; Muroidea; Muridae; Murinae; Mus; Mus
Also known as: 2210416J04Rik; 2310021J10Rik; 2610041P16Rik
Summary: Predicted to enable ubiquinol-cytochrome-c reductase 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; brain; metanephros; respiratory system; and sensory organ. Human ortholog(s) of this gene implicated in mitochondrial complex III deficiency. Orthologous to several human genes including UQCRH (ubiquinol-cytochrome c reductase hinge protein). [provided by Alliance of Genome Resources, Dec 2024]
Expression: Ubiquitous expression in heart adult (RPKM 217.4), bladder adult (RPKM 179.9) and 26 other tissues See more
Orthologs: human all
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Genomic context

Location:: 4 D1; 4 53.1 cM

Exon count:: 4

Annotation release	Status	Assembly	Chr	Location
RS_2024_02	current	GRCm39 (GCF_000001635.27)	4	NC_000070.7 (115924162..115932267, complement)
108.20200622	previous assembly	GRCm38.p6 (GCF_000001635.26)	4	NC_000070.6 (116066965..116075070, complement)

Chromosome 4 - NC_000070.7 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?

AOX delays the onset of the lethal phenotype in a mouse model of Uqcrh (complex III) disease.
Title: AOX delays the onset of the lethal phenotype in a mouse model of Uqcrh (complex III) disease.
Knockout of the Complex III subunit Uqcrh causes bioenergetic impairment and cardiac contractile dysfunction.
Title: Knockout of the Complex III subunit Uqcrh causes bioenergetic impairment and cardiac contractile dysfunction.
Characterising a homozygous two-exon deletion in UQCRH: comparing human and mouse phenotypes.
Title: Characterising a homozygous two-exon deletion in UQCRH: comparing human and mouse phenotypes.

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Variation

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Alleles

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

Targeted (2)

Interactions

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Products	Interactant	Other Gene	Complex	Source	Pubs	Description

General gene information

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Markers

S100a11 (e-PCR), detects polymorphism
- UniSTS:498776

Gene Ontology Provided by MGI

Function	Evidence Code	Pubs
enables protein-containing complex binding	ISO Inferred from Sequence Orthology more info
enables ubiquinol-cytochrome-c reductase activity	ISO Inferred from Sequence Orthology more info
enables ubiquinol-cytochrome-c reductase activity	ISS Inferred from Sequence or Structural Similarity more info

Process	Evidence Code	Pubs
involved_in cellular respiration	NAS Non-traceable Author Statement more info	PubMed
involved_in mitochondrial electron transport, ubiquinol to cytochrome c	IBA Inferred from Biological aspect of Ancestor more info
involved_in mitochondrial electron transport, ubiquinol to cytochrome c	NAS Non-traceable Author Statement more info	PubMed
involved_in proton transmembrane transport	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 inner 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
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

General protein information

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Preferred Names: cytochrome b-c1 complex subunit 6, mitochondrial

Names: complex III subunit 6; complex III subunit VIII; cytochrome C1, nonheme 11 kDa protein; cytochrome c1 non-heme 11 kDa protein; mitochondrial hinge protein; ubiquinol-cytochrome c reductase complex 11 kDa protein

NP_079917.1

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.