Uqcc3 MGI Mouse Gene Detail - MGI:2147553 - ubiquinol-cytochrome c reductase complex assembly factor 3

Symbol

Uqcc3
Name

ubiquinol-cytochrome c reductase complex assembly factor 3
Synonyms

AI462493, Cbp4

Feature Type

protein coding gene
IDs

MGI:2147553
NCBI Gene: 107197
Alliance

genome feature page
Transcription Start Sites

2 TSS

Sequence Map

Chr19:8857378-8858287 bp, - strand
From Ensembl annotation of GRCm39
View this region in JBrowse
Genome Browsers

Ensembl | UCSC | NCBI

Genetic Map

Chromosome 19, 5.90 cM
Mapping Data

1 experiment

SNPs within 2kb

140 from dbSNP Build 142
Strain Annotations

19

For selected strains:

Strain	Gene Model ID	Feature Type	Coordinates
C57BL/6J	MGI_C57BL6J_2147553	protein coding gene	Chr19:8857378-8858297 (-)
129S1/SvImJ	ENSMUSG00200051868	protein coding gene	Chr19:5583104-5584013 (-)
A/J	ENSMUSG00195043998	protein coding gene	Chr19:5984673-5985582 (-)
AKR/J	ENSMUSG00220049737	protein coding gene	Chr19:5757836-5758745 (-)
BALB/cJ	ENSMUSG00180044171	protein coding gene	Chr19:6348651-6349560 (-)
C3H/HeJ	ENSMUSG00175013257	protein coding gene	Chr19:5622398-5623307 (-)
C57BL/6NJ	ENSMUSG00215045363	protein coding gene	Chr19:5823572-5824481 (-)
CAROLI/EiJ	MGP_CAROLIEiJ_G0022661	protein coding gene	Chr19:6085683-6086581 (-)
CAST/EiJ	ENSTCUG00005030746	protein coding gene	Chr19:5460504-5461414 (-)
CBA/J	ENSMUSG00210045056	protein coding gene	Chr19:5638106-5639015 (-)
DBA/2J	ENSMUSG00185051807	protein coding gene	Chr19:5992791-5993700 (-)
FVB/NJ	ENSMUSG00205031613	protein coding gene	Chr19:5680534-5681443 (-)
JF1/MsJ	ENSUMUG00000011424	protein coding gene	Chr19:6229489-6230399 (-)
LP/J	ENSMUSG00230052379	protein coding gene	Chr19:6909757-6910666 (-)
NOD/ShiLtJ	ENSMUSG00190049397	protein coding gene	Chr19:5518146-5519055 (-)
NZO/HlLtJ	ENSMUSG00225045689	protein coding gene	Chr19:10618558-10619467 (-)
PWK/PhJ	ENSLUMG00010005813	protein coding gene	Chr19:5665795-5666705 (-)
SPRET/EiJ	ENSMSPG00010043140	protein coding gene	Chr19:5573182-5573881 (-)
WSB/EiJ	ENSIUOG00005044906	protein coding gene	Chr19:5346906-5347815 (-)

Human Ortholog

UQCC3, ubiquinol-cytochrome c reductase complex assembly factor 3

Vertebrate Orthologs

4

Vertebrate Orthology Source

Alliance of Genome Resources

Human Ortholog

UQCC3, ubiquinol-cytochrome c reductase complex assembly factor 3
Synonyms

C11orf83, CCDS41658.1, MC3DN9, UNQ655
Links

HGNC:34399

NCBI Gene ID: 790955

UniProt: Q6UW78
Chr Location

11q12.3; chr11:62670273-62673686 (+) GRCh38

MGI Vertebrate Homology

Uqcc3 stringent orthology
1 human;1 mouse;2 rat;1 zebrafish
HCOP

vertebrate homology predictions: UQCC3
Gene Tree

Uqcc3

Diseases

1 with human UQCC3 associations

				Human Disease	Mouse Models
				mitochondrial complex III deficiency nuclear type 9 IDs mitochondrial complex III deficiency nuclear type 9 DOID:0080118 OMIM:616111

Click on a disease name to see all genes associated with that disease.

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Phenotype Summary

25 phenotypes from 2 alleles in 2 genetic backgrounds
13 phenotype references

Phenotype Overview

Blue squares indicate phenotypes directly attributed to mutations/alleles of this gene.

adipose tissue	behavior/neurological	cardiovascular system	cellular	craniofacial	digestive/alimentary system	embryo	endocrine/exocrine glands	growth/size/body	hearing/vestibular/ear	hematopoietic system	homeostasis/metabolism	integument	immune system	limbs/digits/tail	liver/biliary system	mortality/aging	muscle	nervous system	pigmentation	renal/urinary system	reproductive system	respiratory system	skeleton	taste/olfaction	neoplasm	vision/eye

Click cells to view annotations.

All Mutations and Alleles

68
Endonuclease-mediated

2
Gene trapped

63
Targeted

3
Find Mice (IMSR)

10 strains or lines available
Comparison Matrix

Gene Expression + Phenotype

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All GO Annotations

27
GO References

6

Molecular Function

carbohydrate derivative binding	cytoskeletal protein binding	DNA binding	enzyme regulator	hydrolase	ligase	lipid binding	oxidoreductase	RNA binding	signaling receptor activity	signaling receptor binding	transcription	transferase	transporter

Biological Process

carbohydrate derivative metabolism	cell differentiation	cell population proliferation	cellular component organization	DNA-templated transcription	establishment of localization	homeostatic process	immune system process	lipid metabolic process	programmed cell death	protein metabolic process	response to stimulus	signaling	system development

Cellular Component

cell projection	cytoplasmic vesicle	cytoskeleton	cytosol	endoplasmic reticulum	endosome	extracellular region	Golgi apparatus	mitochondrion	membraneless organelle	nucleus	organelle envelope	organelle lumen	plasma membrane	protein-containing complex	synapse	vacuole

Click cells to view annotations.

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Expression Overview

GXD's primary emphasis is on endogenous gene expression during development. Click on grid cells to view annotations.

Blue cells = expressed in wild-type.
Gray triangles = other expression annotations only
(e.g. absence of expression or data from mutants).

early conceptus	embryo ectoderm	embryo endoderm	embryo mesoderm	embryo mesenchyme	extraembryonic component	alimentary system	auditory system	branchial arches	cardiovascular system	connective tissue	endocrine system	exocrine system	hemolymphoid system	integumental system	limbs	liver and biliary system	musculoskeletal system	nervous system	olfactory system	reproductive system	respiratory system	urinary system	visual system

Click cells to view annotations.

Assay Results

598
Tissues

1
cDNA Data

63
Literature Summary

1

Tissue x Stage Matrix

Comparison Matrix

Gene Expression + Phenotype

Other Mouse Links

GEO

Expression Atlas
Other Vertebrate Links

Xenbase uqcc3

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All Sequences

38
RefSeq

2
UniProt

2
CCDS

CCDS50383.1

Ensembl

ENSMUSG00000071654 (Evidence)
NCBI Gene

107197

			Length	Strain/Species	Flank
genomic	ENSMUSG00000071654	Ensembl Gene Model \| MGI Sequence Detail	910	C57BL/6J	± kb
transcript	ENSMUST00000096253	Ensembl \| MGI Sequence Detail	821	Not Applicable
polypeptide	ENSMUSP00000093972	Ensembl \| MGI Sequence Detail	89	Not Applicable

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UniProt

2 Sequences
Protein Ontology

PR:000059871 ubiquinol-cytochrome-c reductase complex assembly factor 3

(term hierarchy)
InterPro Domains

IPR027896 Ubiquinol-cytochrome-c reductase complex assembly factor 3
GlyGen

Q8K2T4 1 site

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All nucleic 63

cDNA 63

Microarray probesets 3

Summaries

All 37

Developmental Gene Expression 1

Gene Ontology 6

Phenotypes 13
Earliest

J:86696 Zambrowicz BP, et al., Wnk1 kinase deficiency lowers blood pressure in mice: a gene-trap screen to identify potential targets for therapeutic intervention. Proc Natl Acad Sci U S A. 2003 Nov 25;100(24):14109-14
Latest

J:345621 Adams DJ, et al., Genetic determinants of micronucleus formation in vivo. Nature. 2024 Mar;627(8002):130-136

TSS for Uqcc3:

(View these features in JBrowse)

Transcription Start Site	Location	Distance from Gene 5'-end
Tssr158076	Chr19:8858247-8858292 (-)	17 bp
Tssr158075	Chr19:8858196-8858218 (-)	80 bp

Contributing Projects: Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
Citing These Resources Funding Information Warranty Disclaimer, Privacy Notice, Licensing, & Copyright Send questions and comments to User Support.	last database update 09/08/2026 MGI 6.24