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Tbio
COQ2
4-hydroxybenzoate polyprenyltransferase, mitochondrial

Protein Summary
Description
Catalyzes the prenylation of para-hydroxybenzoate (PHB) with an all-trans polyprenyl group. Mediates the second step in the final reaction sequence of coenzyme Q (CoQ) biosynthesis, which is the condensation of the polyisoprenoid side chain with PHB, generating the first membrane-bound Q intermediate. 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]
Uniprot Accession IDs
Gene Name
Ensembl ID
  • ENST00000311469
  • ENSP00000310873
  • ENSG00000173085
  • ENST00000439031
  • ENSP00000409275

Symbol
  • CL640
  • MSA1
  • CL640
  • COQ10D1
  • PHB:PPT
Illumination Graph
Knowledge Table
Most Knowledge About
Knowledge Value (0 to 1 scale)
kinase perturbation
0.88
transcription factor binding site profile
0.88
transcription factor perturbation
0.74
histone modification site profile
0.73
cellular component
0.64


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: 123.3   (req: < 5)
Gene RIFs: 21   (req: <= 3)
Antibodies: 57   (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: 123.3   (req: >= 5)
Gene RIFs: 21   (req: > 3)
Antibodies: 57   (req: > 50)

- OR - satisfy the following criterion:

Gene Ontology Terms: 6
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
Pathways (9)
Metabolism (R-HSA-1430728)

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Items per page:
1 – 5 of 6
Data Source
Name
Explore in Pharos
Explore in Source
Reactome
Metabolism
Reactome
Metabolism of cofactors
Reactome
Metabolism of vitamins and cofactors
Reactome
Mitochondrial protein import
Reactome
Protein localization
Name
Explore in Pharos
Explore in Source
Metabolism
Metabolism of cofactors
Metabolism of vitamins and cofactors
Mitochondrial protein import
Protein localization
Protein-Protein Interactions (141)
1 – 10 of 141
COQ6
Tbio
Family: Enzyme
Novelty: 0.03821723
Score: 0.995
Data Source: STRINGDB
PDSS1
Tbio
Family: Enzyme
Novelty: 0.01260939
Score: 0.995
Data Source: STRINGDB
PDSS2
Tbio
Family: Enzyme
Novelty: 0.01416723
Score: 0.994
Data Source: STRINGDB
COQ3
Tbio
Family: Enzyme
Novelty: 0.04494536
Score: 0.961
Data Source: STRINGDB
BCS1L
Tbio
Family: Enzyme
Novelty: 0.00258512
Score: 0.96
Data Source: STRINGDB
ATP5F1B
Tbio
Family: Enzyme
Novelty: 0.02087315
Score: 0.955
Data Source: STRINGDB
COQ4
Tbio
Novelty: 0.04287391
Score: 0.937
Data Source: STRINGDB
COQ9
Tbio
Novelty: 0.005822
Score: 0.937
Data Source: STRINGDB
CS
Tbio
Family: Enzyme
Novelty: 0.00182322
Score: 0.934
Data Source: STRINGDB
CYC1
Tbio
Novelty: 0.01978836
Score: 0.934
Data Source: STRINGDB
Publication Statistics
PubMed Score  123.30

PubMed score by year
PubTator Score  90.81

PubTator score by year
Amino Acid Sequence
Residue Counts
Sequence
MLGSRAAGFARGLRALALAWLPGWRGRSFALARAAGAPHGGDLQPPACPEPRGRQLSLSAAAVVDSAPRP
1-70
LQPYLRLMRLDKPIGTWLLYLPCTWSIGLAAEPGCFPDWYMLSLFGTGAILMRGAGCTINDMWDQDYDKK
70-140
VTRTANRPIAAGDISTFQSFVFLGGQLTLALGVLLCLNYYSIALGAGSLLLVITYPLMKRISYWPQLALG
140-210
LTFNWGALLGWSAIKGSCDPSVCLPLYFSGVMWTLIYDTIYAHQDKRDDVLIGLKSTALRFGENTKPWLS
210-280
GFSVAMLGALSLVGVNSGQTAPYYAALGAVGAHLTHQIYTLDIHRPEDCWNKFISNRTLGLIVFLGIVLG
280-350
NLWKEKKTDKTKKGIENKIEN
350-371
MLGSRAAGFARGLRALALAWLPGWRGRSFALARAAGAPHGGDLQPPACPEPRGRQLSLSAAAVVDSAPRPLQPYLRLMRLDKPIGTWLLYLPCTWSIGLAAEPGCFPDWYMLSLFGTGAILMRGAGCTINDMWDQDYDKKVTRTANRPIAAGDISTFQSFVFLGGQLTLALGVLLCLNYYSIALGAGSLLLVITYPLMKRISYWPQLALGLTFNWGALLGWSAIKGSCDPSVCLPLYFSGVMWTLIYDTIYAHQDKRDDVLIGLKSTALRFGENTKPWLSGFSVAMLGALSLVGVNSGQTAPYYAALGAVGAHLTHQIYTLDIHRPEDCWNKFISNRTLGLIVFLGIVLGNLWKEKKTDKTKKGIENKIEN