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Protein

Ubiquinone biosynthesis O-methyltransferase, mitochondrial

Gene

COQ3

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-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

  • glycerol metabolic process Source: UniProtKB
  • regulation of ubiquinone biosynthetic process Source: Ensembl
  • ubiquinone biosynthetic process Source: UniProtKB
Complete GO annotation...

Keywords - Molecular functioni

Methyltransferase, Transferase

Keywords - Biological processi

Ubiquinone biosynthesis

Keywords - Ligandi

S-adenosyl-L-methionine

Enzyme and pathway databases

BioCyciMetaCyc:HS05632-MONOMER.
ZFISH:HS05632-MONOMER.
BRENDAi2.1.1.114. 2681.
2.1.1.222. 2681.
2.1.1.64. 2681.
ReactomeiR-HSA-2142789. Ubiquinol biosynthesis.
UniPathwayiUPA00232.

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

HGNCiHGNC:18175. COQ3.

Subcellular locationi

  • Mitochondrion inner membrane UniRule annotation; Peripheral membrane protein UniRule annotation; Matrix side UniRule annotation

GO - Cellular componenti

  • mitochondrial inner membrane Source: UniProtKB-SubCell
  • mitochondrial matrix Source: Reactome
  • mitochondrion Source: UniProtKB
Complete GO annotation...

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 – 85MitochondrionSequence analysisAdd 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.

PTM databases

iPTMnetiQ9NZJ6.
PhosphoSitePlusiQ9NZJ6.

Expressioni

Gene expression databases

BgeeiENSG00000132423.
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

Protein-protein interaction databases

BioGridi119731. 15 interactors.
IntActiQ9NZJ6. 1 interactor.
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.
OMAiHDWEKFV.
OrthoDBiEOG091G0ICD.
PhylomeDBiQ9NZJ6.
TreeFamiTF314553.

Family and domain databases

Gene3Di3.40.50.150. 1 hit.
HAMAPiMF_00472. UbiG. 1 hit.
InterProiIPR029063. SAM-dependent_MTases.
IPR010233. UbiG_MeTrfase.
[Graphical view]
SUPFAMiSSF53335. SSF53335. 1 hit.
TIGRFAMsiTIGR01983. UbiG. 1 hit.

Sequencei

Sequence statusi: Complete.

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

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

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 → E.2 PublicationsCorresponds to variant rs11548336dbSNPEnsembl.1
Natural variantiVAR_020789272S → G.4 PublicationsCorresponds to variant rs6925344dbSNPEnsembl.1
Natural variantiVAR_020790329Y → H.5 PublicationsCorresponds to variant rs4144164dbSNPEnsembl.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. Translation: CAI17193.1.
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

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. Translation: CAI17193.1.
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. 15 interactors.
IntActiQ9NZJ6. 1 interactor.
STRINGi9606.ENSP00000254759.

PTM databases

iPTMnetiQ9NZJ6.
PhosphoSitePlusiQ9NZJ6.

Polymorphism and mutation databases

BioMutaiCOQ3.
DMDMi313104241.

Proteomic databases

EPDiQ9NZJ6.
MaxQBiQ9NZJ6.
PaxDbiQ9NZJ6.
PeptideAtlasiQ9NZJ6.
PRIDEiQ9NZJ6.

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.
GeneCardsiCOQ3.
H-InvDBHIX0200854.
HGNCiHGNC:18175. COQ3.
HPAiHPA031273.
HPA031274.
MIMi605196. gene.
neXtProtiNX_Q9NZJ6.
OpenTargetsiENSG00000132423.
PharmGKBiPA134934287.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG1270. Eukaryota.
COG2227. LUCA.
GeneTreeiENSGT00390000007284.
HOVERGENiHBG051078.
InParanoidiQ9NZJ6.
KOiK00591.
OMAiHDWEKFV.
OrthoDBiEOG091G0ICD.
PhylomeDBiQ9NZJ6.
TreeFamiTF314553.

Enzyme and pathway databases

UniPathwayiUPA00232.
BioCyciMetaCyc:HS05632-MONOMER.
ZFISH: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.
PROiQ9NZJ6.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000132423.
CleanExiHS_COQ3.
ExpressionAtlasiQ9NZJ6. baseline and differential.
GenevisibleiQ9NZJ6. HS.

Family and domain databases

Gene3Di3.40.50.150. 1 hit.
HAMAPiMF_00472. UbiG. 1 hit.
InterProiIPR029063. SAM-dependent_MTases.
IPR010233. UbiG_MeTrfase.
[Graphical view]
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 historyi
Integrated into UniProtKB/Swiss-Prot: November 2, 2001
Last sequence update: November 30, 2010
Last modified: November 2, 2016
This is version 149 of the entry and version 3 of the sequence. [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. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  6. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.