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Guidelines

MIRAGE

coenzyme Q6, monooxygenase

Summary

Gene Symbol

COQ6

Organism

Homo sapiens (human)

External Links

NCBI Gene

51004

PubChem

51004

Alliance of Genome Resources

HGNC:20233

JoGo

COQ6

TogoVar

COQ6

Annotation

Keyword

Alternative splicing
Cell projection
Deafness
Disease variant
FAD
Golgi apparatus
Mitochondrion inner membrane
Monooxygenase
Primary mitochondrial disease
Proteomics identification
Reference proteome
Transit peptide
Ubiquinone biosynthesis

Proteins

Displaying **all 2** entries
UniProt	Protein Name
Q9Y2Z9	2-methoxy-6-polyprenolphenol 4-hydroxylase Coenzyme Q10 monooxygenase 6
A0A0D9SFJ1

Annotation information from UniProt.

Gene Ontology (GO)

Displaying **all 3** entries
GO Term	Evidence Code	PMID
ubiquinone biosynthetic process	NAS	28927698
ubiquinone biosynthetic process	IBA
ubiquinone biosynthetic process	IEA

GO Hierarchy

biological process

metabolic process [inference]

biosynthetic process [inference]

cellular biosynthetic process [inference]

ketone biosynthetic process [inference]

quinone biosynthetic process [inference]

ubiquinone biosynthetic process

small molecule biosynthetic process [inference]

ketone biosynthetic process [inference]

quinone biosynthetic process [inference]

ubiquinone biosynthetic process

organic substance biosynthetic process [inference]

ketone biosynthetic process [inference]

quinone biosynthetic process [inference]

ubiquinone biosynthetic process

cellular metabolic process [inference]

cellular ketone metabolic process [inference]

ketone biosynthetic process [inference]

quinone biosynthetic process [inference]

ubiquinone biosynthetic process

quinone metabolic process [inference]

ubiquinone metabolic process [inference]

ubiquinone biosynthetic process

quinone biosynthetic process [inference]

ubiquinone biosynthetic process

cellular biosynthetic process [inference]

ketone biosynthetic process [inference]

quinone biosynthetic process [inference]

ubiquinone biosynthetic process

small molecule metabolic process [inference]

cellular ketone metabolic process [inference]

ketone biosynthetic process [inference]

quinone biosynthetic process [inference]

ubiquinone biosynthetic process

quinone metabolic process [inference]

ubiquinone metabolic process [inference]

ubiquinone biosynthetic process

quinone biosynthetic process [inference]

ubiquinone biosynthetic process

small molecule biosynthetic process [inference]

ketone biosynthetic process [inference]

quinone biosynthetic process [inference]

ubiquinone biosynthetic process

organic substance metabolic process [inference]

cellular ketone metabolic process [inference]

ketone biosynthetic process [inference]

quinone biosynthetic process [inference]

ubiquinone biosynthetic process

quinone metabolic process [inference]

ubiquinone metabolic process [inference]

ubiquinone biosynthetic process

quinone biosynthetic process [inference]

ubiquinone biosynthetic process

organic substance biosynthetic process [inference]

ketone biosynthetic process [inference]

quinone biosynthetic process [inference]

ubiquinone biosynthetic process

cellular process [inference]

cellular metabolic process [inference]

cellular ketone metabolic process [inference]

ketone biosynthetic process [inference]

quinone biosynthetic process [inference]

ubiquinone biosynthetic process

quinone metabolic process [inference]

ubiquinone metabolic process [inference]

ubiquinone biosynthetic process

quinone biosynthetic process [inference]

ubiquinone biosynthetic process

cellular biosynthetic process [inference]

ketone biosynthetic process [inference]

quinone biosynthetic process [inference]

ubiquinone biosynthetic process

Displaying entries **1 - 5** of 9 in total

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GO Term	Evidence Code	PMID
mitochondrion	IDA
mitochondrion	IBA
mitochondrion	HTP	34800366
mitochondrial inner membrane	TAS
mitochondrial inner membrane	IDA	27499296

GO Hierarchy

cellular component

protein-containing complex [inference]

ubiquinone biosynthesis complex

cellular anatomical entity [inference]

membrane [inference]

organelle membrane [inference]

organelle inner membrane [inference]

mitochondrial inner membrane

mitochondrial membrane [inference]

mitochondrial inner membrane

extrinsic component of membrane [inference]

extrinsic component of organelle membrane [inference]

extrinsic component of mitochondrial inner membrane

cell projection

organelle [inference]

membrane-bounded organelle [inference]

intracellular membrane-bounded organelle [inference]

intracellular organelle [inference]

intracellular membrane-bounded organelle [inference]

Displaying **all 5** entries
GO Term	Evidence Code	PMID
protein binding	IPI	27499296
oxidoreductase activity	IBA
oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, reduced flavin or flavoprotein as one donor, and incorporation of one atom of oxygen	IEA
FAD binding	IEA
4-hydroxy-3-all-trans-hexaprenylbenzoate oxygenase activity	IEA

GO Hierarchy

molecular function

catalytic activity [inference]

oxidoreductase activity

monooxygenase activity [inference]

oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, reduced flavin or flavoprotein as one donor, and incorporation of one atom of oxygen

4-hydroxy-3-all-trans-hexaprenylbenzoate oxygenase activity

oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen [inference]

oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, reduced flavin or flavoprotein as one donor, and incorporation of one atom of oxygen

4-hydroxy-3-all-trans-hexaprenylbenzoate oxygenase activity

binding [inference]

protein binding

small molecule binding [inference]

heterocyclic compound binding [inference]

nucleotide binding [inference]

flavin adenine dinucleotide binding [inference]

FAD binding

ion binding [inference]

anion binding [inference]

flavin adenine dinucleotide binding [inference]

FAD binding

organic cyclic compound binding [inference]

nucleoside phosphate binding [inference]

nucleotide binding [inference]

flavin adenine dinucleotide binding [inference]

FAD binding

Gene Ontology information from NCBI Gene and UniProt.

OrthoDB (Group)

Group level

Eukaryota

Group Name

ubiquinone biosynthesis

Functional Category

E: Amino acid transport and metabolism
P: Inorganic ion transport and metabolism
Q: Secondary metabolites biosynthesis, transport and catabolism

Displaying entries **1 - 10** of 12 in total

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Gene Ontology
2-octaprenyl-6-methoxyphenol hydroxylase activity
2-octaprenylphenol hydroxylase activity
3-demethoxyubiquinol 3-hydroxylase activity
FAD binding
carotene biosynthetic process
flavin adenine dinucleotide binding
monooxygenase activity
oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, NAD(P)H as one donor, and incorporation of one atom of oxygen
oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen
oxidoreductase activity

Displaying **all 5** entries
InterPro
FAD-binding domain
FAD/NAD(P)-binding domain superfamily
Ubiquinone biosynthesis hydroxylase UbiH/COQ6
Ubiquinone biosynthesis hydroxylase, UbiH/UbiF/VisC/COQ6, conserved site
Ubiquinone biosynthesis monooxygenase COQ6

Disease

Disease Ontology

Displaying 1 entry
DO ID	Disease Name	Source
DOID:0070243	primary coenzyme Q10 deficiency 6	Alliance of Genome Resources

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Supported by JST NBDC Grant Number JPMJND2204

Partly supported by NIH Common Fund Grant #1U01GM125267-01

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Last updated: April 6, 2026