Tbio COQ6 Ubiquinone biosynthesis monooxygenase COQ6, mitochondrial

Protein Summary

Description

FAD-dependent monooxygenase required for the C5-ring hydroxylation during ubiquinone biosynthesis. Catalyzes the hydroxylation of 3-polyprenyl-4-hydroxybenzoic acid to 3-polyprenyl-4,5-dihydroxybenzoic acid. The electrons required for the hydroxylation reaction may be funneled indirectly from NADPH via a ferredoxin/ferredoxin reductase system to COQ6. The protein encoded by this gene belongs to the ubiH/COQ6 family. It is an evolutionarily conserved monooxygenase required for the biosynthesis of coenzyme Q10 (or ubiquinone), which is an essential component of the mitochondrial electron transport chain, and one of the most potent lipophilic antioxidants implicated in the protection of cell damage by reactive oxygen species. Knockdown of this gene in mouse and zebrafish results in decreased growth due to increased apoptosis. Mutations in this gene are associated with autosomal recessive coenzyme Q10 deficiency-6 (COQ10D6), which manifests as nephrotic syndrome with sensorineural deafness. Alternatively spliced transcript variants encoding different isoforms have been described for this gene. [provided by RefSeq, Jun 2012]

Uniprot Accession IDs

• Q9Y2Z9
• B7Z3K8
• Q53GG6
• Q86U30
• Q96CA1
• Q96CK2

Gene Name

• COQ6

Ensembl ID

• ENST00000334571
• ENSP00000333946
• ENSG00000119723
• ENST00000394026
• ENSP00000377594

Symbol

• CGI10
• CGI-10
• COQ10D6

Knowledge Table

Most Knowledge About	Knowledge Value (0 to 1 scale)
virus perturbation	0.98
histone modification site profile	0.75
protein domain	0.73
transcription factor perturbation	0.68
gene perturbation	0.65

Protein Classes

PANTHER Classes

Protein / Oxidoreductase / Oxygenase / Ubiquinone Biosynthesis Monooxygenase Coq6, Mitochondrial

DTO Classes

Protein / Enzyme / Oxidoreductase / Oxygenase / Ubiquinone Biosynthesis Monooxygenase Coq6, Mitochondrial

IDG Development Level Summary

Tdark

These are targets about which virtually nothing is known. They do not have known drug or small molecule activities
- AND - satisfy two or more of the following criteria:

Pubmed score: 29.25   (req: < 5)

Gene RIFs: 6   (req: <= 3)

Antibodies: 167   (req: <= 50)

Tbio

These targets do not have known drug or small molecule activities
- AND - satisfy two or more of the following criteria:

Pubmed score: 29.25   (req: >= 5)

Gene RIFs: 6   (req: > 3)

Antibodies: 167   (req: > 50)

- OR - satisfy the following criterion:

Gene Ontology Terms: 4

Tchem

Target has at least one ChEMBL compound with an activity cutoff of < 30 nM - AND - satisfies the preceding conditions

Active Ligand: 0

Tclin

Target has at least one approved drug - AND - satisfies the preceding conditions

Active Drug: 0