Homo sapiens Gene: COQ2
Summary
InnateDB Gene IDBG-27632.6
Last Modified 2014-10-13 [Report errors or provide feedback]
Gene Symbol COQ2
Gene Name coenzyme Q2 homolog, prenyltransferase (yeast)
Synonyms
Species Homo sapiens
Ensembl Gene ENSG00000173085
Encoded Proteins
coenzyme Q2 homolog, prenyltransferase (yeast)
coenzyme Q2 homolog, prenyltransferase (yeast)
coenzyme Q2 homolog, prenyltransferase (yeast)
coenzyme Q2 homolog, prenyltransferase (yeast)
Protein Structure
Useful resources Stemformatics EHFPI ImmGen
Entrez Gene
Summary This gene encodes an enzyme that functions in the final steps in the biosynthesis of CoQ (ubiquinone), a redox carrier in the mitochondrial respiratory chain and a lipid-soluble antioxidant. This enzyme, which is part of the coenzyme Q10 pathway, catalyzes the prenylation of parahydroxybenzoate with an all-trans polyprenyl group. Mutations in this gene cause coenzyme Q10 deficiency, a mitochondrial encephalomyopathy, and also COQ2 nephropathy, an inherited form of mitochondriopathy with primary renal involvement. [provided by RefSeq, Oct 2009]
Gene Information
Type Protein coding
Genomic Location Chromosome 4:83261536-83284914
Strand Reverse strand
Band q21.23
Transcripts
ENST00000311469 ENSP00000310873
ENST00000311461 ENSP00000311835
ENST00000503391 ENSP00000426242
ENST00000503915 ENSP00000427146
ENST00000514935
Interactions
Number of Interactions This gene and/or its encoded proteins are associated with 1 experimentally validated interaction(s) in this database.
Experimentally validated
Total 1 [view]
Protein-Protein 1 [view]
Protein-DNA 0
Protein-RNA 0
DNA-DNA 0
RNA-RNA 0
DNA-RNA 0
Gene Ontology

Molecular Function
Accession GO Term
GO:0002083 4-hydroxybenzoate decaprenyltransferase activity
GO:0004659 prenyltransferase activity
GO:0047293 4-hydroxybenzoate nonaprenyltransferase activity
Biological Process
GO:0006071 glycerol metabolic process
GO:0006744 ubiquinone biosynthetic process
GO:0008152 metabolic process
GO:0008219 cell death
GO:0008299 isoprenoid biosynthetic process
GO:0009058 biosynthetic process
GO:0044281 small molecule metabolic process
Cellular Component
GO:0005739 mitochondrion
GO:0005743 mitochondrial inner membrane
GO:0016021 integral component of membrane
Orthologs
Species
Mus musculus
Bos taurus
Gene ID
Gene Order
Not yet available
Pathways
NETPATH
REACTOME
Mitochondrial protein import pathway
Ubiquinol biosynthesis pathway
Metabolism of lipids and lipoproteins pathway
Metabolism of proteins pathway
Metabolism pathway
KEGG
Ubiquinone and other terpenoid-quinone biosynthesis pathway
INOH
PID NCI
Cross-References
SwissProt
TrEMBL E7EPM7
UniProt Splice Variant
Entrez Gene 27235
UniGene Hs.729069
RefSeq NM_015697 XM_005262927
HUGO HGNC:25223
OMIM 609825
CCDS CCDS47090
HPRD 13062
IMGT
EMBL AC114781
GenPept
RNA Seq Atlas 27235