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Protein

Ubiquinone biosynthesis O-methyltransferase, mitochondrial

Gene

COQ3

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

O-methyltransferase that catalyzes the 2 O-methylation steps in the ubiquinone biosynthetic pathway.UniRule annotation

Catalytic activityi

S-adenosyl-L-methionine + 3,4-dihydroxy-5-all-trans-polyprenylbenzoate = S-adenosyl-L-homocysteine + 3-methoxy-4-hydroxy-5-all-trans-polyprenylbenzoate.UniRule annotation1 Publication
S-adenosyl-L-methionine + 3-demethylubiquinone-n = S-adenosyl-L-homocysteine + ubiquinone-n.UniRule annotation1 Publication

Pathwayi: ubiquinone biosynthesis

This protein is involved in the pathway ubiquinone biosynthesis, which is part of Cofactor biosynthesis.UniRule annotation
View all proteins of this organism that are known to be involved in the pathway ubiquinone biosynthesis and in Cofactor biosynthesis.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Binding sitei124S-adenosyl-L-methionineUniRule annotation1
Binding sitei154S-adenosyl-L-methionine; via carbonyl oxygenUniRule annotation1
Binding sitei175S-adenosyl-L-methionineUniRule annotation1
Binding sitei222S-adenosyl-L-methionine; via carbonyl oxygenUniRule annotation1

GO - Molecular functioni

GO - Biological processi

Keywordsi

Molecular functionMethyltransferase, Transferase
Biological processUbiquinone biosynthesis
LigandS-adenosyl-L-methionine

Enzyme and pathway databases

BioCyciMetaCyc:HS05632-MONOMER
BRENDAi2.1.1.114 2681
2.1.1.222 2681
2.1.1.64 2681
ReactomeiR-HSA-2142789 Ubiquinol biosynthesis
UniPathwayi
UPA00232

Names & Taxonomyi

Protein namesi
Recommended name:
Ubiquinone biosynthesis O-methyltransferase, mitochondrialUniRule annotation
Alternative name(s):
3-demethylubiquinol 3-O-methyltransferaseUniRule annotation (EC:2.1.1.64UniRule annotation)
Polyprenyldihydroxybenzoate methyltransferaseUniRule annotation (EC:2.1.1.114UniRule annotation)
Gene namesi
Name:COQ3UniRule annotation
ORF Names:UG0215E05
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 6

Organism-specific databases

EuPathDBiHostDB:ENSG00000132423.10
HGNCiHGNC:18175 COQ3
MIMi605196 gene
neXtProtiNX_Q9NZJ6

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion inner membrane

Pathology & Biotechi

Organism-specific databases

DisGeNETi51805
OpenTargetsiENSG00000132423
PharmGKBiPA134934287

Polymorphism and mutation databases

BioMutaiCOQ3
DMDMi313104241

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 85MitochondrionUniRule annotationAdd BLAST85
ChainiPRO_000003592686 – 369Ubiquinone biosynthesis O-methyltransferase, mitochondrialAdd BLAST284

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei143N6-acetyllysineBy similarity1
Modified residuei149N6-acetyllysineBy similarity1
Modified residuei196N6-acetyllysineBy similarity1

Keywords - PTMi

Acetylation

Proteomic databases

EPDiQ9NZJ6
MaxQBiQ9NZJ6
PaxDbiQ9NZJ6
PeptideAtlasiQ9NZJ6
PRIDEiQ9NZJ6
ProteomicsDBi83417

PTM databases

iPTMnetiQ9NZJ6
PhosphoSitePlusiQ9NZJ6

Expressioni

Gene expression databases

BgeeiENSG00000132423 Expressed in 207 organ(s), highest expression level in biceps brachii
CleanExiHS_COQ3
ExpressionAtlasiQ9NZJ6 baseline and differential
GenevisibleiQ9NZJ6 HS

Organism-specific databases

HPAiHPA031273
HPA031274

Interactioni

Subunit structurei

Component of a multi-subunit COQ enzyme complex, composed of at least COQ3, COQ4, COQ5, COQ6, COQ7 and COQ9.UniRule annotation

Binary interactionsi

Protein-protein interaction databases

BioGridi119731, 16 interactors
IntActiQ9NZJ6, 29 interactors
STRINGi9606.ENSP00000254759

Structurei

3D structure databases

ProteinModelPortaliQ9NZJ6
SMRiQ9NZJ6
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the class I-like SAM-binding methyltransferase superfamily. UbiG/COQ3 family.UniRule annotation

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG1270 Eukaryota
COG2227 LUCA
GeneTreeiENSGT00390000007284
HOVERGENiHBG051078
InParanoidiQ9NZJ6
KOiK00591
OMAiGTHDWEK
OrthoDBiEOG091G0ICD
PhylomeDBiQ9NZJ6
TreeFamiTF314553

Family and domain databases

HAMAPiMF_00472 UbiG, 1 hit
InterProiView protein in InterPro
IPR029063 SAM-dependent_MTases
IPR010233 UbiG_MeTrfase
SUPFAMiSSF53335 SSF53335, 1 hit
TIGRFAMsiTIGR01983 UbiG, 1 hit

Sequence (1+)i

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

This entry has 1 described isoform and 1 potential isoform that is computationally mapped.Show allAlign All

Q9NZJ6-1 [UniParc]FASTAAdd to basket
« Hide
        10         20         30         40         50
MWSGRKLGSS GGWFLRVLGP GGCNTKAARP LISSAVYVKN QLSGTLQIKP
60 70 80 90 100
GVFNEYRTIW FKSYRTIFSC LNRIKSFRYP WARLYSTSQT TVDSGEVKTF
110 120 130 140 150
LALAHKWWDE QGVYAPLHSM NDLRVPFIRD NLLKTIPNHQ PGKPLLGMKI
160 170 180 190 200
LDVGCGGGLL TEPLGRLGAS VIGIDPVDEN IKTAQCHKSF DPVLDKRIEY
210 220 230 240 250
RVCSLEEIVE ETAETFDAVV ASEVVEHVID LETFLQCCCQ VLKPGGSLFI
260 270 280 290 300
TTINKTQLSY ALGIVFSEQI ASIVPKGTHT WEKFVSPETL ESILESNGLS
310 320 330 340 350
VQTVVGMLYN PFSGYWHWSE NTSLNYAAYA VKSRVQEHPA SAEFVLKGET
360
EELQANACTN PAVHEKLKK
Length:369
Mass (Da):41,054
Last modified:November 30, 2010 - v3
Checksum:iB257A099D6C06A2D
GO

Computationally mapped potential isoform sequencesi

There is 1 potential isoform mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
F6WGP0F6WGP0_HUMAN
Ubiquinone biosynthesis O-methyltra...
COQ3
141Annotation score:

Sequence cautioni

The sequence AAF66826 differs from that shown. Reason: Frameshift at positions 16, 18, 22, 26, 44, 49, 60, 363 and 364.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti21G → V in CAB66660 (PubMed:11230166).Curated1
Sequence conflicti180N → D in CAB66660 (PubMed:11230166).Curated1
Sequence conflicti287 – 289PET → LEP in AAN76515 (Ref. 1) Curated3
Sequence conflicti321N → D in AAN76515 (Ref. 1) Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_061925134K → E2 PublicationsCorresponds to variant dbSNP:rs11548336Ensembl.1
Natural variantiVAR_020789272S → G4 PublicationsCorresponds to variant dbSNP:rs6925344Ensembl.1
Natural variantiVAR_020790329Y → H5 PublicationsCorresponds to variant dbSNP:rs4144164Ensembl.1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF351615 mRNA Translation: AAN76515.1
AL136726 mRNA Translation: CAB66660.1
AK056955 mRNA Translation: BAG51832.1
AL513550 Genomic DNA No translation available.
BC015634 mRNA Translation: AAH15634.2
BC063463 mRNA Translation: AAH63463.1
AF193016 mRNA Translation: AAF66826.1 Frameshift.
CCDSiCCDS5042.1
RefSeqiNP_059117.3, NM_017421.3
UniGeneiHs.713623

Genome annotation databases

EnsembliENST00000254759; ENSP00000254759; ENSG00000132423
GeneIDi51805
KEGGihsa:51805
UCSCiuc003ppk.4 human

Keywords - Coding sequence diversityi

Polymorphism

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF351615 mRNA Translation: AAN76515.1
AL136726 mRNA Translation: CAB66660.1
AK056955 mRNA Translation: BAG51832.1
AL513550 Genomic DNA No translation available.
BC015634 mRNA Translation: AAH15634.2
BC063463 mRNA Translation: AAH63463.1
AF193016 mRNA Translation: AAF66826.1 Frameshift.
CCDSiCCDS5042.1
RefSeqiNP_059117.3, NM_017421.3
UniGeneiHs.713623

3D structure databases

ProteinModelPortaliQ9NZJ6
SMRiQ9NZJ6
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi119731, 16 interactors
IntActiQ9NZJ6, 29 interactors
STRINGi9606.ENSP00000254759

PTM databases

iPTMnetiQ9NZJ6
PhosphoSitePlusiQ9NZJ6

Polymorphism and mutation databases

BioMutaiCOQ3
DMDMi313104241

Proteomic databases

EPDiQ9NZJ6
MaxQBiQ9NZJ6
PaxDbiQ9NZJ6
PeptideAtlasiQ9NZJ6
PRIDEiQ9NZJ6
ProteomicsDBi83417

Protocols and materials databases

DNASUi51805
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000254759; ENSP00000254759; ENSG00000132423
GeneIDi51805
KEGGihsa:51805
UCSCiuc003ppk.4 human

Organism-specific databases

CTDi51805
DisGeNETi51805
EuPathDBiHostDB:ENSG00000132423.10
GeneCardsiCOQ3
H-InvDBiHIX0200854
HGNCiHGNC:18175 COQ3
HPAiHPA031273
HPA031274
MIMi605196 gene
neXtProtiNX_Q9NZJ6
OpenTargetsiENSG00000132423
PharmGKBiPA134934287
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG1270 Eukaryota
COG2227 LUCA
GeneTreeiENSGT00390000007284
HOVERGENiHBG051078
InParanoidiQ9NZJ6
KOiK00591
OMAiGTHDWEK
OrthoDBiEOG091G0ICD
PhylomeDBiQ9NZJ6
TreeFamiTF314553

Enzyme and pathway databases

UniPathwayi
UPA00232

BioCyciMetaCyc:HS05632-MONOMER
BRENDAi2.1.1.114 2681
2.1.1.222 2681
2.1.1.64 2681
ReactomeiR-HSA-2142789 Ubiquinol biosynthesis

Miscellaneous databases

GenomeRNAii51805
PROiPR:Q9NZJ6
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000132423 Expressed in 207 organ(s), highest expression level in biceps brachii
CleanExiHS_COQ3
ExpressionAtlasiQ9NZJ6 baseline and differential
GenevisibleiQ9NZJ6 HS

Family and domain databases

HAMAPiMF_00472 UbiG, 1 hit
InterProiView protein in InterPro
IPR029063 SAM-dependent_MTases
IPR010233 UbiG_MeTrfase
SUPFAMiSSF53335 SSF53335, 1 hit
TIGRFAMsiTIGR01983 UbiG, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiCOQ3_HUMAN
AccessioniPrimary (citable) accession number: Q9NZJ6
Secondary accession number(s): B3KPX0
, Q5T061, Q6P4F0, Q8IXG6, Q96BG1, Q9H0N1
Entry historyiIntegrated into UniProtKB/Swiss-Prot: November 2, 2001
Last sequence update: November 30, 2010
Last modified: October 10, 2018
This is version 159 of the entry and version 3 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. Human chromosome 6
    Human chromosome 6: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. SIMILARITY comments
    Index of protein domains and families
  4. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  5. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  6. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
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Main funding by: National Institutes of Health

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