Coq4 MGI Mouse Gene Detail - MGI:1098826

Symbol

Coq4
Name

coenzyme Q4
Synonyms

D2Ertd97e, EST-MNCb4625

Feature Type

protein coding gene
IDs

MGI:1098826
NCBI Gene: 227683
Alliance

genome feature page
Transcription Start Sites

4 TSS

Sequence Map

Chr2:29677505-29687947 bp, + strand
From NCBI annotation of GRCm39
View this region in JBrowse
Genome Browsers

Ensembl | UCSC | NCBI

Genetic Map

Chromosome 2, 20.62 cM
Mapping Data

2 experiments

SNPs within 2kb

328 from dbSNP Build 142
Strain Annotations

19

For selected strains:

Strain	Gene Model ID	Feature Type	Coordinates
C57BL/6J	MGI_C57BL6J_1098826	protein coding gene	Chr2:29677505-29687947 (+)
129S1/SvImJ	ENSMUSG00200047826	protein coding gene	Chr2:26647613-26658074 (+)
A/J	ENSMUSG00195037953	protein coding gene	Chr2:26741280-26751722 (+)
AKR/J	ENSMUSG00220049379	protein coding gene	Chr2:26859819-26870261 (+)
BALB/cJ	ENSMUSG00180051571	protein coding gene	Chr2:26760569-26771011 (+)
C3H/HeJ	ENSMUSG00175052020	protein coding gene	Chr2:26729888-26740329 (+)
C57BL/6NJ	ENSMUSG00215048763	protein coding gene	Chr2:26795559-26806000 (+)
CAROLI/EiJ	MGP_CAROLIEiJ_G0023370	protein coding gene	Chr2:25648447-25658951 (+)
CAST/EiJ	ENSTCUG00005052638	protein coding gene	Chr2:26466005-26476657 (+)
CBA/J	ENSMUSG00210016573	protein coding gene	Chr2:26763046-26773486 (+)
DBA/2J	ENSMUSG00185046178	protein coding gene	Chr2:26655359-26665801 (+)
FVB/NJ	ENSMUSG00205044166	protein coding gene	Chr2:26507385-26517827 (+)
JF1/MsJ	ENSUMUG00000021261	protein coding gene	Chr2:26551725-26562212 (+)
LP/J	ENSMUSG00230036842	protein coding gene	Chr2:28583387-28593848 (+)
NOD/ShiLtJ	ENSMUSG00190038520	protein coding gene	Chr2:26762203-26772645 (+)
NZO/HlLtJ	ENSMUSG00225007297	protein coding gene	Chr2:36332429-36342905 (+)
PWK/PhJ	ENSLUMG00010042734	protein coding gene	Chr2:26628885-26639355 (+)
SPRET/EiJ	ENSMSPG00010042621	protein coding gene	Chr2:27351829-27362469 (+)
WSB/EiJ	ENSIUOG00005050358	protein coding gene	Chr2:26614104-26624565 (+)

Human Ortholog

COQ4, coenzyme Q4

Vertebrate Orthologs

3

Vertebrate Orthology Source

Alliance of Genome Resources

Human Ortholog

COQ4, coenzyme Q4
Synonyms

CGI-92, COQ10D7, SPAX10
Links

HGNC:19693

NCBI Gene ID: 51117

UniProt: Q9Y3A0
Chr Location

9q34.11; chr9:128322515-128334072 (+) GRCh38

MGI Vertebrate Homology

Coq4 stringent orthology
1 human;1 mouse;1 rat;1 zebrafish
HCOP

vertebrate homology predictions: COQ4
Gene Tree

Coq4

Diseases

2 with human COQ4 associations

				Human Disease	Mouse Models
				primary coenzyme Q10 deficiency 7 IDs primary coenzyme Q10 deficiency 7 DOID:0070244 OMIM:616276 ORDO:457185
				spastic ataxia IDs spastic ataxia DOID:0050952 MESH:C564815

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

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Phenotype Summary

5 phenotypes from 1 allele in 1 genetic background
10 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

14
Endonuclease-mediated

1
Gene trapped

11
Targeted

2
Incidental Mutations

Mutagenetix , APF
Find Mice (IMSR)

18 strains or lines available
Comparison Matrix

Gene Expression + Phenotype

Mice homozygous for a knock-out allele exhibit defects in chorion formation and placentation, abnormal trophoblast layer morphology, and complete lethality throughout fetal growth and development.

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All GO Annotations

30
GO References

14

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

631
Tissues

19
cDNA Data

32
Literature Summary

3

Images

Tissue x Stage Matrix

Comparison Matrix

Gene Expression + Phenotype

Other Mouse Links

Allen Institute

GEO

Expression Atlas
Other Vertebrate Links

Xenbase coq4

ZFIN coq4

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All Sequences

63
RefSeq

9
UniProt

6
CCDS

CCDS15857.1

Ensembl

ENSMUSG00000026798 (Evidence)
NCBI Gene

227683

			Length	Strain/Species	Flank
genomic	227683	NCBI Gene Model \| MGI Sequence Detail	10443	C57BL/6J	± kb
transcript	NR_145503	RefSeq \| MGI Sequence Detail	3026	C57BL/6
polypeptide	Q8BGB8	UniProt \| EBI \| MGI Sequence Detail	266	Not Applicable

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UniProt

6 Sequences
Protein Ontology

PR:000005753 ubiquinone biosynthesis protein COQ4, mitochondrial

(term hierarchy)
EC

4.1.1.130
InterPro Domains

IPR007715 Ubiquinone biosynthesis protein Coq4

IPR027540 Ubiquinone biosynthesis protein Coq4, eukaryotic

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All nucleic 35

cDNA 32

Primer pair 2

Other 1

Antibodies 1

Microarray probesets 4

Summaries

All 50

Developmental Gene Expression 3

Gene Ontology 14

Phenotypes 10
Earliest

J:60984 Ko MS, et al., Large-scale cDNA analysis reveals phased gene expression patterns during preimplantation mouse development. Development. 2000 Apr;127(8):1737-49
Latest

J:360510 Pelosi L, et al., COQ4 is required for the oxidative decarboxylation of the C1 carbon of coenzyme Q in eukaryotic cells. Mol Cell. 2024 Mar 7;84(5):981-989.e7

TSS for Coq4:

(View these features in JBrowse)

Transcription Start Site	Location	Distance from Gene 5'-end
Tssr17018	Chr2:29677494-29677523 (+)	4 bp
Tssr17019	Chr2:29678249-29678314 (+)	777 bp
Tssr17020	Chr2:29678320-29678332 (+)	821 bp
Tssr17021	Chr2:29678333-29678347 (+)	835 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