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COQ3 coenzyme Q3, methyltransferase [ Homo sapiens (human) ]

Gene ID: 51805, updated on 7-Jun-2020

Summary

Official Symbol
COQ3provided by HGNC
Official Full Name
coenzyme Q3, methyltransferaseprovided by HGNC
Primary source
HGNC:HGNC:18175
See related
Ensembl:ENSG00000132423 MIM:605196
Gene type
protein coding
RefSeq status
VALIDATED
Organism
Homo sapiens
Lineage
Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae; Homo
Also known as
DHHBMT; bA9819.1; DHHBMTASE; UG0215E05
Summary
Ubiquinone, also known as coenzyme Q, or Q, is a critical component of the electron transport pathways of both eukaryotes and prokaryotes (Jonassen and Clarke, 2000 [PubMed 10777520]). This lipid consists of a hydrophobic isoprenoid tail and a quinone head group. The tail varies in length depending on the organism, but its purpose is to anchor coenzyme Q to the membrane. The quinone head group is responsible for the activity of coenzyme Q in the respiratory chain. The S. cerevisiae COQ3 gene encodes an O-methyltransferase required for 2 steps in the biosynthetic pathway of coenzyme Q. This enzyme methylates an early coenzyme Q intermediate, 3,4-dihydroxy-5-polyprenylbenzoic acid, as well as the final intermediate in the pathway, converting demethyl-ubiquinone to coenzyme Q. The COQ3 gene product is also capable of methylating the distinct prokaryotic early intermediate 2-hydroxy-6-polyprenyl phenol.[supplied by OMIM, Mar 2008]
Expression
Ubiquitous expression in heart (RPKM 8.1), brain (RPKM 3.9) and 25 other tissues See more
Orthologs

Genomic context

See COQ3 in Genome Data Viewer
Location:
6q16.2
Exon count:
8
Annotation release Status Assembly Chr Location
109.20200522 current GRCh38.p13 (GCF_000001405.39) 6 NC_000006.12 (99369401..99394223, complement)
105 previous assembly GRCh37.p13 (GCF_000001405.25) 6 NC_000006.11 (99817279..99842082, complement)

Chromosome 6 - NC_000006.12Genomic Context describing neighboring genes Neighboring gene 3-hydroxybutyrate dehydrogenase 2, pseudogene 1 Neighboring gene failed axon connections homolog, metaxin like GST domain containing Neighboring gene uncharacterized LOC101927365 Neighboring gene PNN interacting serine and arginine rich protein Neighboring gene thiosulfate sulfurtransferase like domain containing 3 Neighboring gene ubiquitin specific peptidase 45

Genomic regions, transcripts, and products

Expression

  • Project title: HPA RNA-seq normal tissues
  • Description: RNA-seq was performed of tissue samples from 95 human individuals representing 27 different tissues in order to determine tissue-specificity of all protein-coding genes
  • BioProject: PRJEB4337
  • Publication: PMID 24309898
  • Analysis date: Wed Apr 4 07:08:55 2018

Pathways from PubChem

Interactions

Products Interactant Other Gene Complex Source Pubs Description

General gene information

Markers

Homology

Gene Ontology Provided by GOA

Function Evidence Code Pubs
2-polyprenyl-6-methoxy-1,4-benzoquinone methyltransferase activity IEA
Inferred from Electronic Annotation
more info
 
3-demethylubiquinone-10 3-O-methyltransferase activity TAS
Traceable Author Statement
more info
 
3-demethylubiquinone-9 3-O-methyltransferase activity IEA
Inferred from Electronic Annotation
more info
 
O-methyltransferase activity IGI
Inferred from Genetic Interaction
more info
PubMed 
decaprenyldihydroxybenzoate methyltransferase activity TAS
Traceable Author Statement
more info
 
hexaprenyldihydroxybenzoate methyltransferase activity IEA
Inferred from Electronic Annotation
more info
 
protein binding IPI
Inferred from Physical Interaction
more info
PubMed 
Process Evidence Code Pubs
glycerol metabolic process IGI
Inferred from Genetic Interaction
more info
PubMed 
methylation IEA
Inferred from Electronic Annotation
more info
 
regulation of ubiquinone biosynthetic process IEA
Inferred from Electronic Annotation
more info
 
ubiquinone biosynthetic process IEA
Inferred from Electronic Annotation
more info
 
ubiquinone biosynthetic process IGI
Inferred from Genetic Interaction
more info
PubMed 
ubiquinone biosynthetic process TAS
Traceable Author Statement
more info
 
Component Evidence Code Pubs
extrinsic component of mitochondrial inner membrane IEA
Inferred from Electronic Annotation
more info
 
mitochondrial matrix TAS
Traceable Author Statement
more info
 
mitochondrion IDA
Inferred from Direct Assay
more info
PubMed 

General protein information

Preferred Names
ubiquinone biosynthesis O-methyltransferase, mitochondrial
Names
2-polyprenyl-6-hydroxyphenol methylase
2-polyprenyl-6-hydroxyphenyl methylase
3,4-dihydroxy-5-hexaprenylbenzoate methyltransferase
3-demethylubiquinol 3-O-methyltransferase
3-demethylubiquinone-10 3-methyltransferase
DHHB methyltransferase
DHHB-MTase
coenzyme Q3 homolog, methyltransferase
dihydroxyhexaprenylbenzoate methyltransferase
hexaprenyldihydroxybenzoate methyltransferase, mitochondrial
methyltransferase COQ3
polyprenyldihydroxybenzoate methyltransferase
NP_059117.3
XP_005267075.1
XP_006715563.1
XP_011534200.1
XP_011534201.1
XP_024302225.1
XP_024302226.1

NCBI Reference Sequences (RefSeq)

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_017421.4NP_059117.3  ubiquinone biosynthesis O-methyltransferase, mitochondrial

    See identical proteins and their annotated locations for NP_059117.3

    Status: VALIDATED

    Source sequence(s)
    AL136726, AL513550, BC001534
    Consensus CDS
    CCDS5042.1
    UniProtKB/Swiss-Prot
    Q9NZJ6
    Related
    ENSP00000254759.3, ENST00000254759.8
    Conserved Domains (2) summary
    TIGR01983
    Location:96326
    UbiG; ubiquinone biosynthesis O-methyltransferase
    pfam08241
    Location:151250
    Methyltransf_11; Methyltransferase domain

RefSeqs of Annotated Genomes: Homo sapiens Updated Annotation Release 109.

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

Reference GRCh38.p13 Primary Assembly

Genomic

  1. NC_000006.12 Reference GRCh38.p13 Primary Assembly

    Range
    99369401..99394223 complement
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. XM_006715500.3XP_006715563.1  ubiquinone biosynthesis O-methyltransferase, mitochondrial isoform X1

    Conserved Domains (3) summary
    TIGR01983
    Location:137367
    UbiG; ubiquinone biosynthesis O-methyltransferase
    pfam08241
    Location:192291
    Methyltransf_11; Methyltransferase domain
    pfam13900
    Location:84121
    GVQW; Putative domain of unknown function
  2. XM_024446458.1XP_024302226.1  ubiquinone biosynthesis O-methyltransferase, mitochondrial isoform X4

    Conserved Domains (1) summary
    cl28104
    Location:1207
    UbiG; 2-polyprenyl-3-methyl-5-hydroxy-6-metoxy-1,4-benzoquinol methylase [Coenzyme transport and metabolism]
  3. XM_024446457.1XP_024302225.1  ubiquinone biosynthesis O-methyltransferase, mitochondrial isoform X4

    Conserved Domains (1) summary
    cl28104
    Location:1207
    UbiG; 2-polyprenyl-3-methyl-5-hydroxy-6-metoxy-1,4-benzoquinol methylase [Coenzyme transport and metabolism]
  4. XM_011535899.1XP_011534201.1  ubiquinone biosynthesis O-methyltransferase, mitochondrial isoform X4

    Conserved Domains (1) summary
    cl28104
    Location:1207
    UbiG; 2-polyprenyl-3-methyl-5-hydroxy-6-metoxy-1,4-benzoquinol methylase [Coenzyme transport and metabolism]
  5. XM_011535898.2XP_011534200.1  ubiquinone biosynthesis O-methyltransferase, mitochondrial isoform X2

    Conserved Domains (2) summary
    TIGR01983
    Location:137286
    UbiG; ubiquinone biosynthesis O-methyltransferase
    pfam13900
    Location:84121
    GVQW; Putative domain of unknown function
  6. XM_005267018.4XP_005267075.1  ubiquinone biosynthesis O-methyltransferase, mitochondrial isoform X3

    Conserved Domains (1) summary
    TIGR01983
    Location:96245
    UbiG; ubiquinone biosynthesis O-methyltransferase
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