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

Gene ID: 70383, updated on 27-Nov-2024

Summary

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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

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See Cox10 in Genome Data Viewer
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_000077.7Genomic Context describing neighboring genes Neighboring gene STARR-seq mESC enhancer starr_29740 Neighboring gene heparan sulfate (glucosamine) 3-O-sulfotransferase 3B1 Neighboring gene STARR-positive B cell enhancer ABC_E1420 Neighboring gene STARR-seq mESC enhancer starr_29745 Neighboring gene STARR-seq mESC enhancer starr_29746 Neighboring gene RIKEN cDNA 9630013K17 gene Neighboring gene predicted gene, 30060 Neighboring gene predicted gene 12289 Neighboring gene predicted gene, 51866 Neighboring gene RIKEN cDNA 2810001G20 gene Neighboring gene STARR-seq mESC enhancer starr_29751 Neighboring gene STARR-seq mESC enhancer starr_29752 Neighboring gene STARR-seq mESC enhancer starr_29754 Neighboring gene predicted gene 12290

Genomic regions, transcripts, and products

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Go to nucleotide: Graphics FASTA GenBank

Expression

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  • 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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Related articles in PubMed

  1. Sustained AMPK activation improves muscle function in a mitochondrial myopathy mouse model by promoting muscle fiber regeneration. Peralta S, et al. Hum Mol Genet, 2016 Aug 1. PMID 27288451, Free PMC Article
  2. Cytochrome c Oxidase Activity Is a Metabolic Checkpoint that Regulates Cell Fate Decisions During T Cell Activation and Differentiation. Tarasenko TN, et al. Cell Metab, 2017 Jun 6. PMID 28591633, Free PMC Article
  3. Deletion of the Mitochondrial Complex-IV Cofactor Heme A:Farnesyltransferase Causes Focal Segmental Glomerulosclerosis and Interferon Response. Baek JH, et al. Am J Pathol, 2018 Dec. PMID 30268775, Free PMC Article
  4. Cytochrome c oxidase deficiency in neurons decreases both oxidative stress and amyloid formation in a mouse model of Alzheimer's disease. Fukui H, et al. Proc Natl Acad Sci U S A, 2007 Aug 28. PMID 17715058, Free PMC Article
  5. Mitochondrial myopathy induces a starvation-like response. Tyynismaa H, et al. Hum Mol Genet, 2010 Oct 15. PMID 20656789

See all (56) citations in PubMed

GeneRIFs: Gene References Into Functions

What's a GeneRIF?
  1. 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.
  2. 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.
  3. 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.
  4. 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.
  5. 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.
Submit: New GeneRIF Correction

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

Pathways from PubChem

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General gene information

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Markers

Homology

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 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
XP_006534231.1

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)

  1. 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

  1. NC_000077.7 Reference GRCm39 C57BL/6J

    Range
    63853453..63970298 complement
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. 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
Nucleotide Protein Strain
Heading Accession and Version
Protein Accession Links
GenPept Link UniProtKB Link
Q8CFY5.1 GenPept UniProtKB/Swiss-Prot:Q8CFY5

Gene LinkOut

The following LinkOut resources are supplied by external providers. These providers are responsible for maintaining the links.

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