COQ2

Gene Information

Official Symbol: COQ2
Official Name: coenzyme Q2, polyprenyltransferase
Aliases and Previous Symbols: N/A
Entrez ID: 27235
UniProt: Q96H96
Interactions: BioGRID
PubMed articles: Open PubMed
OMIM: Open OMIM

Function Summary

Entrez Summary: 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 Summary: 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. {ECO:0000255|HAMAP-Rule:MF_03189, ECO:0000269|PubMed:15153069, ECO:0000269|PubMed:16400613, ECO:0000269|PubMed:17374725, ECO:0000269|PubMed:27493029}.

Pfam Domains GO Terms

CRISPR Data

Compound Hit Most Correlated Genes in Chemogenomics Tissues where Essential in the Avana Dataset (DepMap 20Q1)

Gene	Correlation
NDUFAF7	0.591
GTPBP10	0.572
RPUSD4	0.57
COA6	0.564
MRPS34	0.563
C12orf65	0.561
NDUFA3	0.561
MRPL23	0.555
MRPL21	0.551
VARS2	0.549
MRPL53	0.548
COQ3	0.544
RNASEH1	0.543
NDUFA2	0.538
SELRC1	0.536
SCO2	0.536
PDSS1	0.535
MRPL47	0.534
FASTKD2	0.532
NDUFAF3	0.531
COQ4	0.53
NDUFC2	0.529
NDUFB11	0.528
LRPPRC	0.525
TARS2	0.523
GFM1	0.522
MRPS23	0.522
MRPS21	0.521
GFM2	0.517
COQ6	0.515
COQ7	0.515
MRPL55	0.515
MTRF1L	0.51
MRP63	0.51
FTSJ2	0.509
MTG2	0.508
ACAD9	0.508
PET117	0.507
DAP3	0.503
EARS2	0.502
MRPS25	0.502
MRPS5	0.501
WBSCR16	0.501
TEFM	0.5
RNMTL1	0.5
MRPS14	0.499
BCS1L	0.498
PNPT1	0.498
TIMM17A	0.497
SARS2	0.497
YARS2	0.496
FASTKD5	0.495
LARS2	0.494
NDUFS7	0.493
MTIF3	0.493
PDSS2	0.493
UQCC1	0.491
MRPL44	0.49
QRSL1	0.49
COX16	0.489
MRPL15	0.489
NDUFB3	0.489
AARS2	0.488
PARS2	0.488
MRPS10	0.487
MRPS16	0.487
SUPV3L1	0.484
GRSF1	0.484
MRPS6	0.484
MTO1	0.484
HSD17B10	0.483
NARS2	0.482
NDUFS2	0.48
COQ5	0.48
POLG2	0.479
COA3	0.477
TIMM8B	0.476
MRPS18A	0.474
MRPL1	0.471
COX11	0.47
CHCHD1	0.469
MRPL12	0.468
NDUFB5	0.468
ERAL1	0.467
MRPS11	0.467
TMEM261	0.466
METTL17	0.466
KIAA0391	0.464
IBA57	0.464
MTG1	0.463
NDUFB9	0.462
TIMMDC1	0.461
MRPL45	0.461
MRPS24	0.46
POLG	0.46
NDUFV1	0.459
MRPL3	0.459
NUBPL	0.458
CARS2	0.457
COX6C	0.456
ECSIT	0.456
C17orf89	0.455
UQCRC2	0.454
SLC25A26	0.454
PET112	0.454
MRPS9	0.453
MRPL4	0.453
MTERFD2	0.452
NDUFB2	0.452
MRPS12	0.452
NDUFAF4	0.452
RBFA	0.451
HMGXB3	0.451
MRPL22	0.45
DDX28	0.45
MCAT	0.448
NDUFA1	0.447
NDUFAF6	0.446
WARS2	0.446
NDUFA11	0.446
NDUFC1	0.444
RPUSD3	0.444
MRPL20	0.442
MTPAP	0.442
TUFM	0.44
PITRM1	0.44
NDUFB7	0.439
AASDHPPT	0.438
NDUFS5	0.438
NDUFS8	0.437
FARS2	0.436
MIPEP	0.434
NDUFA8	0.433
MRPS26	0.433
FASTKD1	0.43
ICT1	0.428
RTN4IP1	0.426
MRPS7	0.425
NDUFB6	0.425
MRPL13	0.424
C10orf2	0.421
TOMM20	0.42
MTIF2	0.419
GLRX5	0.418
UQCC2	0.418
COX15	0.417
RARS2	0.417
DHX30	0.415
MRPL43	0.413
MARS2	0.413
GATC	0.413
COX6B1	0.411
MRPL39	0.409
TRUB2	0.409
MRPS27	0.407
MRPL18	0.407
MALSU1	0.405
SSBP1	0.404
DARS2	0.404
HARS2	0.402
ATP5SL	0.402
RMND1	0.401
PTCD3	0.401
MRPS22	0.4
FOXRED1	0.4
MTERFD1	0.4
TOMM22	0.4

Global Fraction of Cell Lines Where Essential: 10/739

Tissue	Fraction Of Cell Lines Where Essential
1290807.0	0/1
909776.0	0/1
bile duct	1/28
blood	1/28
bone	0/26
breast	1/33
central nervous system	1/56
cervix	0/4
colorectal	0/17
esophagus	0/13
fibroblast	0/1
gastric	1/16
kidney	1/21
liver	0/20
lung	1/75
lymphocyte	0/16
ovary	0/26
pancreas	0/24
peripheral nervous system	0/16
plasma cell	0/15
prostate	0/1
skin	0/24
soft tissue	0/9
thyroid	0/2
upper aerodigestive	1/22
urinary tract	0/29
uterus	0/5

Essentiality in NALM6

Essentiality Rank: 1978
Expression level (log2 read counts): 3.99

Expression Distribution

Screen	Score
2-Deoxy-D-glucose 800μM R05 exp214	-1.87
UM0125461 0.74μM R02 exp84	1.7
Ammonium tetrathiomolybdate 10μM R08 exp448	3.14

4-hydroxybenzoate nonaprenyltransferase activity
4-hydroxybenzoate decaprenyltransferase activity
transferase activity, transferring alkyl or aryl (other than methyl) groups
quinone biosynthetic process
ubiquinone biosynthetic process
ubiquinone metabolic process
glycerol metabolic process
alditol metabolic process
isoprenoid biosynthetic process
ketone biosynthetic process
quinone metabolic process
integral component of mitochondrial inner membrane
oxidoreduction coenzyme metabolic process
cellular ketone metabolic process
polyol metabolic process
coenzyme biosynthetic process
isoprenoid metabolic process
cellular carbohydrate metabolic process
cofactor biosynthetic process
coenzyme metabolic process
alcohol metabolic process
mitochondrial inner membrane
cofactor metabolic process
organic hydroxy compound metabolic process
carbohydrate metabolic process
lipid biosynthetic process
small molecule biosynthetic process
cellular lipid metabolic process
lipid metabolic process
mitochondrion
small molecule metabolic process