Gene Ontology annotation through association of InterPro records with GO terms

Annotation inferences using phylogenetic trees

Gene Ontology annotation based on UniProtKB/Swiss-Prot Subcellular Location vocabulary mapping, accompanied by conservative changes to GO terms applied by UniProt

GO_REF:0000052

Gene Ontology annotation based on curation of immunofluorescence data

GO_REF:0000104

Electronic Gene Ontology annotations created by transferring manual GO annotations between related proteins based on shared sequence features

GO_REF:0000120

Combined Automated Annotation using Multiple IEA Methods

PMID:25152161

Molecular characterization of the human COQ5 C-methyltransferase in coenzyme Q10 biosynthesis.

COQ5 catalyzes the only C-methylation in coenzyme Q biosynthesis and is a component of the CoQ synthome; the human polypeptide localizes to the matrix side of the mitochondrial inner membrane and partially complements yeast coq5 mutants.

PMID:27499296

Mitochondrial Protein Interaction Mapping Identifies Regulators of Respiratory Chain Function.

Interaction-mapping study that identified COQ5 within the COQ enzyme complex and provided IDA/IPI evidence for its inner-membrane localization and synthome membership.

PMID:28927698

Biochemistry of Mitochondrial Coenzyme Q Biosynthesis.

Review of mitochondrial coenzyme Q biosynthesis describing the COQ synthome and the role of COQ5 as the C-methyltransferase.

PMID:34800366

Quantitative high-confidence human mitochondrial proteome and its dynamics in cellular context.

COQ5 detected in the high-confidence human mitochondrial proteome.

PMID:35614220

Defining mitochondrial protein functions through deep multiomic profiling.

Deep multiomic profiling identified the direct PYURF-COQ5 interaction; PYURF stabilizes COQ5 and associates with the COQ enzyme complex. Also supports inner-membrane localization of COQ5.

PMID:38425362

In vitro construction of the COQ metabolon unveils the molecular determinants of coenzyme Q biosynthesis.

In vitro reconstruction of the animal COQ metabolon showed that COQ5 performs the C2 methylation step using SAM (kcat 2.4 min-1, KM 444 uM) and co-elutes with COQ3/4/6/7/9 as a complex.

PMID:29044765

A novel inborn error of the coenzyme Q10 biosynthesis pathway: cerebellar ataxia and static encephalomyopathy due to COQ5 C-methyltransferase deficiency.

Biallelic COQ5 variants cause primary coenzyme Q10 deficiency (COQ10D9) with cerebellar ataxia and static encephalomyopathy.

file:human/COQ5/COQ5-uniprot.txt

UniProt entry Q5HYK3 (COQ5_HUMAN)

RecName 2-methoxy-6-polyprenyl-1,4-benzoquinol methylase (EC 2.1.1.201); SAM-dependent C-methyltransferase of the class I MenG/UbiE family; mitochondrial inner membrane, matrix side; component of the multi-subunit COQ enzyme complex; ubiquinone biosynthesis pathway.

Reactome:R-HSA-2142789

Ubiquinol biosynthesis

Reactome:R-HSA-2162186

COQ3 methylates DeMQ10H2

Reactome:R-HSA-2162187

COQ6 hydroxylates DHB

Reactome:R-HSA-2162188

COQ5 methylates MDMQ10H2

Reactome:R-HSA-2162193

COQ3 methylates DHDB

Reactome:R-HSA-2162194

COQ7:COQ9 octamer hydroxylates DMQ10H2

Reactome:R-HSA-2162195

COQ4 decarboxylates MHDB