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LMP002529

Record overview

LMPD IDLMP002529
Gene ID51805
SpeciesHomo sapiens (Human)
Gene Namecoenzyme Q3 methyltransferase
Gene SymbolCOQ3
SynonymsDHHBMT; DHHBMTASE; UG0215E05; bA9819.1
Alternate nameshexaprenyldihydroxybenzoate 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
Chromosome6
Map Location6q16.2
EC Number2.1.1.114
SummaryUbiquinone, 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]
OrthologsView 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
MWSGRKLGSSGGWFLRVLGPGGCNTKAARPLISSAVYVKNQLSGTLQIKPGVFNEYRTIWFKSYRTIFSCLNRIKSFRYPWARLYSTSQTTVDSGEVKTF
LALAHKWWDEQGVYAPLHSMNDLRVPFIRDNLLKTIPNHQPGKPLLGMKILDVGCGGGLLTEPLGRLGASVIGIDPVDENIKTAQCHKSFDPVLDKRIEY
RVCSLEEIVEETAETFDAVVASEVVEHVIDLETFLQCCCQVLKPGGSLFITTINKTQLSYALGIVFSEQIASIVPKGTHTWEKFVSPETLESILESNGLS
VQTVVGMLYNPFSGYWHWSENTSLNYAAYAVKSRVQEHPASAEFVLKGETEELQANACTNPAVHEKLKK