Gene/Proteome Database (LMPD)

LMPD ID
LMP002529
Gene ID
51805
Species
Homo sapiens (Human)
Gene Name
coenzyme Q3 methyltransferase
Gene Symbol
COQ3
Synonyms
DHHBMT; DHHBMTASE; UG0215E05; bA9819.1
Alternate Names
hexaprenyldihydroxybenzoate methyltransferase, mitochondrial; DHHB-MT; DHHB-MTase; DHHB methyltransferase; methyltransferase COQ3; 2-polyprenyl-6-hydroxyphenol methylase; 2-polyprenyl-6-hydroxyphenyl methylase; coenzyme Q3 homolog, methyltransferase; 3-demethylubiquinone-10 3-methyltransferase; dihydroxyhexaprenylbenzoate methyltransferase; polyprenyldihydroxybenzoate methyltransferase; 3,4-dihydroxy-5-hexaprenylbenzoate methyltransferase
Chromosome
6
Map Location
6q16.2
EC Number
2.1.1.114
Summary
Ubiquinone, also known as coenzyme Q, or Q, is a critical component of the electron transport pathways of both eukaryotes and prokaryotes (Jonassen and Clarke, 2000 [PubMed 10777520]). This lipid consists of a hydrophobic isoprenoid tail and a quinone head group. The tail varies in length depending on the organism, but its purpose is to anchor coenzyme Q to the membrane. The quinone head group is responsible for the activity of coenzyme Q in the respiratory chain. The S. cerevisiae COQ3 gene encodes an O-methyltransferase required for 2 steps in the biosynthetic pathway of coenzyme Q. This enzyme methylates an early coenzyme Q intermediate, 3,4-dihydroxy-5-polyprenylbenzoic acid, as well as the final intermediate in the pathway, converting demethyl-ubiquinone to coenzyme Q. The COQ3 gene product is also capable of methylating the distinct prokaryotic early intermediate 2-hydroxy-6-polyprenyl phenol.[supplied by OMIM, Mar 2008]
Orthologs
View orthologs and multiple alignments for COQ3

Proteins

hexaprenyldihydroxybenzoate methyltransferase, mitochondrial
Refseq ID NP_059117
Protein GI 118600965
UniProt ID Q9NZJ6
mRNA ID NM_017421
Length 369
RefSeq Status VALIDATED
MWSGRKLGSSGGWFLRVLGPGGCNTKAARPLISSAVYVKNQLSGTLQIKPGVFNEYRTIWFKSYRTIFSCLNRIKSFRYPWARLYSTSQTTVDSGEVKTFLALAHKWWDEQGVYAPLHSMNDLRVPFIRDNLLKTIPNHQPGKPLLGMKILDVGCGGGLLTEPLGRLGASVIGIDPVDENIKTAQCHKSFDPVLDKRIEYRVCSLEEIVEETAETFDAVVASEVVEHVIDLETFLQCCCQVLKPGGSLFITTINKTQLSYALGIVFSEQIASIVPKGTHTWEKFVSPETLESILESNGLSVQTVVGMLYNPFSGYWHWSENTSLNYAAYAVKSRVQEHPASAEFVLKGETEELQANACTNPAVHEKLKK

Gene Information

Entrez Gene ID
51805
Gene Name
coenzyme Q3 methyltransferase
Gene Symbol
COQ3
Species
Homo sapiens

Gene Ontology (GO Annotations)

GO ID Source Type Description
GO:0005759 TAS:Reactome C mitochondrial matrix
GO:0005739 IDA:UniProtKB C mitochondrion
GO:0008425 IEA:InterPro F 2-polyprenyl-6-methoxy-1,4-benzoquinone methyltransferase activity
GO:0008689 IEA:UniProtKB-EC F 3-demethylubiquinone-9 3-O-methyltransferase activity
GO:0008171 IGI:UniProtKB F O-methyltransferase activity
GO:0004395 IEA:UniProtKB-EC F hexaprenyldihydroxybenzoate methyltransferase activity
GO:0006071 IGI:UniProtKB P glycerol metabolic process
GO:0044281 TAS:Reactome P small molecule metabolic process
GO:0006744 IGI:UniProtKB P ubiquinone biosynthetic process

KEGG Pathway Links

KEGG Pathway ID Description
hsa01100 Metabolic pathways
hsa00130 Ubiquinone and other terpenoid-quinone biosynthesis

REACTOME Pathway Links

REACTOME Pathway ID Description
REACT_160111 Ubiquinol biosynthesis

Domain Information

InterPro Annotations

Accession Description
IPR013216 Methyltransferase type 11
IPR029063 S-adenosyl-L-methionine-dependent methyltransferase
IPR010233 Ubiquinone biosynthesis O-methyltransferase

UniProt Annotations

Entry Information

Gene Name
coenzyme Q3 methyltransferase
Protein Entry
COQ3_HUMAN
UniProt ID
Q9NZJ6
Species
Human

Comments

Comment Type Description
Catalytic Activity S-adenosyl-L-methionine + 3,4-dihydroxy-5-all- trans-polyprenylbenzoate = S-adenosyl-L-homocysteine + 3-methoxy- 4-hydroxy-5-all-trans-polyprenylbenzoate.
Catalytic Activity S-adenosyl-L-methionine + 3- demethylubiquinone-n = S-adenosyl-L-homocysteine + ubiquinone-n.
Catalytic Activity S-adenosyl-L-methionine + 3-(all-trans- polyprenyl)benzene-1,2-diol = S-adenosyl-L-homocysteine + 2- methoxy-6-(all-trans-polyprenyl)phenol.
Pathway Cofactor biosynthesis; ubiquinone biosynthesis.
Sequence Caution Sequence=AAF66826.1; Type=Frameshift; Positions=16, 18, 22, 26, 44, 49, 60, 363, 364; Evidence= ;
Similarity Belongs to the methyltransferase superfamily. UbiG/COQ3 family.
Subcellular Location Mitochondrion matrix .

Identical and Related Proteins

Unique RefSeq proteins for LMP002529 (as displayed in Record Overview)

Protein GI Database Accession Length Protein Name
118600965 RefSeq NP_059117 369 hexaprenyldihydroxybenzoate methyltransferase, mitochondrial

Identical Sequences to LMP002529 proteins

Reference Database Accession Length Protein Name
GI:118600965 SwissProt Q9NZJ6.3 369 RecName: Full=Hexaprenyldihydroxybenzoate methyltransferase, mitochondrial; AltName: Full=2-polyprenyl-6-hydroxyphenol methylase; AltName: Full=3,4-dihydroxy-5-hexaprenylbenzoate methyltransferase; Short=DHHB methyltransferase; Short=DHHB-MT; Short=DHHB-MTase; AltName: Full=3-demethylubiquinone-10 3-methyltransferase; AltName: Full=Dihydroxyhexaprenylbenzoate methyltransferase; Flags: Precursor [Homo sapiens]

Related Sequences to LMP002529 proteins

Reference Database Accession Length Protein Name
GI:118600965 DBBJ BAJ20869.1 369 coenzyme Q3 homolog, methyltransferase, partial [synthetic construct]
GI:118600965 GenBank AAH63463.1 369 Coenzyme Q3 homolog, methyltransferase (S. cerevisiae) [Homo sapiens]
GI:118600965 GenBank ABE15334.1 369 Sequence 2867 from patent US 6979557
GI:118600965 GenBank EAW48482.1 369 coenzyme Q3 homolog, methyltransferase (yeast) [Homo sapiens]
GI:118600965 GenBank ADQ32547.1 369 coenzyme Q3 homolog, methyltransferase (S. cerevisiae), partial [synthetic construct]
GI:118600965 GenBank AIC56506.1 369 COQ3, partial [synthetic construct]