Cox10 heme A:farnesyltransferase cytochrome c oxidase assembly factor 10 [Mus musculus (house mouse)] - Gene

Summary

Official Symbol: Cox10provided by MGI
Official Full Name: heme A:farnesyltransferase cytochrome c oxidase assembly factor 10provided by MGI
Primary source: MGI:MGI:1917633
See related: Ensembl:ENSMUSG00000042148 AllianceGenome:MGI:1917633
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: 2410004F01Rik
Summary: Enables protoheme IX farnesyltransferase activity. Involved in heme O biosynthetic process. Acts upstream of or within several processes, including copper ion homeostasis; heme A biosynthetic process; and mitochondrial fission. Located in mitochondrion. Is active in mitochondrial matrix. Is expressed in alimentary system; nervous system; respiratory system; and submandibular gland primordium. Used to study Parkinson's disease; cytochrome-c oxidase deficiency disease; and mitochondrial myopathy. Human ortholog(s) of this gene implicated in mitochondrial complex IV deficiency nuclear type 3. Orthologous to human COX10 (cytochrome c oxidase assembly factor heme A:farnesyltransferase COX10). [provided by Alliance of Genome Resources, Nov 2024]
Expression: Ubiquitous expression in heart adult (RPKM 18.8), kidney adult (RPKM 13.0) and 28 other tissues See more
Orthologs: human all
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Genomic context

Location:: 11 B3; 11 39.47 cM

Exon count:: 8

Annotation release	Status	Assembly	Chr	Location
RS_2024_02	current	GRCm39 (GCF_000001635.27)	11	NC_000077.7 (63853453..63970298, complement)
108.20200622	previous assembly	GRCm38.p6 (GCF_000001635.26)	11	NC_000077.6 (63962627..64079472, complement)

Chromosome 11 - NC_000077.7 Genomic Context describing neighboring genes

Genomic regions, transcripts, and products

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Go to reference sequence details

Genomic Sequence:

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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?

Reliance on Cox10 and oxidative metabolism for antigen-specific NK cell expansion.
Title: Reliance on Cox10 and oxidative metabolism for antigen-specific NK cell expansion.
miR-144/451 represses mitochondrial respiration during erythropoiesis by inhibiting the production of Cox10.
Title: Regulation of gene expression by miR-144/451 during mouse erythropoiesis.
Loss of COX10 is associated with severe, early-onset Focal Segmental Glomerulosclerosis with innate immune activation and kidney failure.
Title: Deletion of the Mitochondrial Complex-IV Cofactor Heme A:Farnesyltransferase Causes Focal Segmental Glomerulosclerosis and Interferon Response.
The levels of respiratory complex I were markedly reduced in COX10 knockout clones.
Title: Cytochrome c oxidase is required for the assembly/stability of respiratory complex I in mouse fibroblasts.
Mice lacking COX10 in skeletal muscle recapitulate the phenotype of progressive mitochondrial myopathies associated with cytochrome c oxidase deficiency.
Title: Mice lacking COX10 in skeletal muscle recapitulate the phenotype of progressive mitochondrial myopathies associated with cytochrome c oxidase deficiency.

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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 (1) 1 citation

General gene information

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Markers

AU042636 (e-PCR)
- UniSTS:180229

AW549955 (e-PCR)
- UniSTS:208692

NoName (e-PCR)
- UniSTS:236610

NoName (e-PCR)
- UniSTS:236611

NoName (e-PCR)
- UniSTS:236612

D11Mit339 (e-PCR), detects polymorphism
- UniSTS:126556

44.MMHAP54FLG5.seq (e-PCR)
- UniSTS:160105

Cox10 (e-PCR), detects polymorphism
- UniSTS:546703

G54543 (e-PCR)
- UniSTS:117681

Cox10 (e-PCR), detects polymorphism
- UniSTS:527290

Gene Ontology Provided by MGI

Function	Evidence Code	Pubs
enables farnesyltranstransferase activity	ISO Inferred from Sequence Orthology more info
enables protoheme IX farnesyltransferase activity	IBA Inferred from Biological aspect of Ancestor more info
enables protoheme IX farnesyltransferase activity	IMP Inferred from Mutant Phenotype more info	PubMed
enables protoheme IX farnesyltransferase activity	ISO Inferred from Sequence Orthology more info	PubMed

Process	Evidence Code	Pubs
acts_upstream_of_or_within aerobic respiration	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within copper ion homeostasis	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within cytochrome complex assembly	IDA Inferred from Direct Assay more info	PubMed
involved_in heme A biosynthetic process	IBA Inferred from Biological aspect of Ancestor more info
acts_upstream_of_or_within heme A biosynthetic process	IDA Inferred from Direct Assay more info	PubMed
involved_in heme A biosynthetic process	IGI Inferred from Genetic Interaction more info	PubMed
acts_upstream_of_or_within heme A biosynthetic process	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in heme A biosynthetic process	ISO Inferred from Sequence Orthology more info
involved_in heme O biosynthetic process	IGI Inferred from Genetic Interaction more info	PubMed
acts_upstream_of_or_within leukocyte differentiation	IMP Inferred from Mutant Phenotype more info	PubMed
involved_in lipid metabolic process	IEA Inferred from Electronic Annotation more info
acts_upstream_of_or_within mitochondrial fission	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within mitochondrion organization	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within multicellular organism growth	IMP Inferred from Mutant Phenotype more info	PubMed
acts_upstream_of_or_within protein farnesylation	ISO Inferred from Sequence Orthology more info
involved_in respiratory chain complex IV assembly	IEA Inferred from Electronic Annotation more info
involved_in respiratory chain complex IV assembly	ISO Inferred from Sequence Orthology more info

Component	Evidence Code	Pubs
part_of cytochrome complex	IEA Inferred from Electronic Annotation more info
part_of cytochrome complex	ISO Inferred from Sequence Orthology more info
located_in cytosol	IEA Inferred from Electronic Annotation more info
located_in cytosol	ISO Inferred from Sequence Orthology more info
is_active_in mitochondrial matrix	IDA Inferred from Direct Assay more info	PubMed
located_in mitochondrial membrane	IEA Inferred from Electronic Annotation more info
located_in mitochondrion	HDA more info	PubMed
is_active_in mitochondrion	IBA Inferred from Biological aspect of Ancestor more info
located_in mitochondrion	IDA Inferred from Direct Assay more info	PubMed
located_in mitochondrion	ISO Inferred from Sequence Orthology more info
located_in nucleolus	IEA Inferred from Electronic Annotation more info
located_in nucleolus	ISO Inferred from Sequence Orthology more info

General protein information

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Preferred Names: protoheme IX farnesyltransferase, mitochondrial

Names: COX10 homolog, cytochrome c oxidase assembly protein, heme A: farnesyltransferase; cytochrome c oxidase assembly protein 10; heme O synthase

NP_848466.1

EC 2.5.1.141

XP_006534231.1

EC 2.5.1.141

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_178379.3 → NP_848466.1 protoheme IX farnesyltransferase, mitochondrial

See identical proteins and their annotated locations for NP_848466.1

Status: VALIDATED

Source sequence(s)

AK149999, AL603889, BY078593

Consensus CDS

CCDS24839.1

UniProtKB/Swiss-Prot

Q8CFY5

UniProtKB/TrEMBL

Q3TAS4, Q3U3N1

Related

ENSMUSP00000040138.9, ENSMUST00000049091.9

Conserved Domains (1) summary

cd13957
Location:159 → 414

PT_UbiA_Cox10; Protoheme IX farnesyltransferase

RefSeqs of Annotated Genomes: GCF_000001635.27-RS_2024_02

The following sections contain reference sequences that belong to a specific genome build. Explain

Reference GRCm39 C57BL/6J

Genomic

NC_000077.7 Reference GRCm39 C57BL/6J

Range

63853453..63970298 complement

Download

GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

XM_006534168.4 → XP_006534231.1 protoheme IX farnesyltransferase, mitochondrial isoform X1

Conserved Domains (1) summary

cd13957
Location:1 → 198

PT_UbiA_Cox10; Protoheme IX farnesyltransferase