Proteins/UNIPROT:Q9Y3A0

COQ4

ProteinUNIPROT:Q9Y3A0

Lyase that catalyzes the C1-decarboxylation of 4-hydroxy-3-methoxy-5-(all-trans-decaprenyl)benzoic acid into 2-methoxy-6-(all-trans-decaprenyl)phenol during ubiquinone biosynthesis..

Function from UniProtKB (Q9Y3A0), read 2026-09-23. CC BY 4.0.

Curated compounds routed: 0

What the reference databases state

GenCC submitters' classifications

2 rows

mitochondrial disease has a germline basis in COQ4DiseaseGenCC HGNC:19693|MONDO:0044970Inferred only
neonatal encephalomyopathy-cardiomyopathy-respiratory distress syndrome has a germline basis in COQ4DiseaseGenCC HGNC:19693|MONDO:0014562Inferred only

Statements

28 statements

28 statements · 4 reference databases · Subject: COQ4 · Sources: Hetionet, Reactome, Rhea, UniProt

Order

UBC interacts with COQ4ProteinHetionet hetionet:interacts:51117-7316Inferred only
COQ4 participates in MetabolismPathwayReactome R-HSA-1430728Inferred only
COQ4 participates in Metabolism of cofactorsPathwayReactome R-HSA-8978934Inferred only
COQ4 participates in Metabolism of vitamins and cofactorsPathwayReactome R-HSA-196854Inferred only
COQ4 participates in Ubiquinol biosynthesisPathwayReactome R-HSA-2142789Inferred only
ATP6V1D increases the expression of COQ4ProteinHetionet hetionet:regulates:51382-51117Inferred only
BPHL increases the expression of COQ4ProteinHetionet hetionet:regulates:670-51117Inferred only
CD58 increases the expression of COQ4ProteinHetionet hetionet:regulates:965-51117Inferred only
CDK11B decreases the expression of COQ4ProteinHetionet hetionet:regulates:984-51117Inferred only
CHN1 increases the expression of COQ4ProteinHetionet hetionet:regulates:1123-51117Inferred only
CSK decreases the expression of COQ4ProteinHetionet hetionet:regulates:1445-51117Inferred only
DEK increases the expression of COQ4ProteinHetionet hetionet:regulates:7913-51117Inferred only

Show the remaining 16

GNAS increases the expression of COQ4ProteinHetionet hetionet:regulates:2778-51117Inferred only
INPPL1 increases the expression of COQ4ProteinHetionet hetionet:regulates:3636-51117Inferred only
LIG1 decreases the expression of COQ4ProteinHetionet hetionet:regulates:3978-51117Inferred only
LSP1 decreases the expression of COQ4ProteinHetionet hetionet:regulates:4046-51117Inferred only
MRPS2 decreases the expression of COQ4ProteinHetionet hetionet:regulates:51116-51117Inferred only
NFYB increases the expression of COQ4ProteinHetionet hetionet:regulates:4801-51117Inferred only
PGBD1 decreases the expression of COQ4ProteinHetionet hetionet:regulates:84547-51117Inferred only
PON3 increases the expression of COQ4ProteinHetionet hetionet:regulates:5446-51117Inferred only
RB1 decreases the expression of COQ4ProteinHetionet hetionet:regulates:5925-51117Inferred only
SIK1 decreases the expression of COQ4ProteinHetionet hetionet:regulates:150094-51117Inferred only
SLC35A3 increases the expression of COQ4ProteinHetionet hetionet:regulates:23443-51117Inferred only
SLC35F2 increases the expression of COQ4ProteinHetionet hetionet:regulates:54733-51117Inferred only
SRSF2 decreases the expression of COQ4ProteinHetionet hetionet:regulates:6427-51117Inferred only
COQ4 requires the cofactor Zn(2+)CompoundUniProt Q9Y3A0Inferred only
2-methoxy-6-(all-trans-decaprenyl)phenol is a substrate of COQ4CompoundRhea RHEA:81275Inferred only
4-hydroxy-3-methoxy-5-(all-trans-decaprenyl)benzoate is a substrate of COQ4CompoundRhea RHEA:81275Inferred only

Reactions

1 reaction

4-hydroxy-3-methoxy-5-(all-trans-decaprenyl)benzoate+hydron2-methoxy-6-(all-trans-decaprenyl)phenol+carbon dioxide
Catalysed by COQ4
Reaction pageDirection not assertedBalanced
Rhea RHEA:81275CC BY 4.0Reference data

Other names

2 names

Resolves to: COQ4

Also called: 4-hydroxy-3-methoxy-5-polyprenylbenzoate decarboxylaseCoenzyme Q biosynthesis protein 4 homolog

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