COQ3 Gene Summary [Human]

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]

Details

Type
Protein Coding
Official Symbol
COQ3
Official Name
coenzyme Q3, methyltransferase [Source:HGNC Symbol;Acc:HGNC:18175]
Ensembl ID
ENSG00000132423
Bio databases IDs NCBI: 51805 Ensembl: ENSG00000132423
Aliases coenzyme Q3, methyltransferase, polyprenyldihydroxybenzoate methyltransferase
Synonyms 4732433J24, bA9819.1, coenzyme Q3 methyltransferase, coenzyme Q3, methyltransferase, DHHBMT, DHHBMTASE, UG0215E05
Species
Human, Homo sapiens
OrthologiesMouseRat

Protein Domains

A protein domain is a distinct structural or functional region within a protein that can evolve, function, and exist independently of the rest of the protein chain. These domains in human COQ3 often fold into stable, three-dimensional structures and are associated with specific biological functions, such as binding to DNA, other proteins, or small molecules.
  • ubiquinone biosynthesis O-methyltransferase
  • O-methyltransferase
  • 3-demethylubiquinone-9 3-O-methyltransferase
  • hexaprenyldihydroxybenzoate methyltransferase
  • enzyme
  • protein binding
  • AdoMet_MTases
  • S-adenosylmethionine-dependent methyltransferase
  • methyltransferase domain

Top Findings

The most significant associations for this gene, including commonly observed domains, pathway involvement, and functional highlights based on current data.
binds
regulated by
regulates
  • glycerol
  • ubiquinone
  • ubiquinone 6
role in cell
  • growth

Subcellular Expression

Locations within the cell where the protein is known or predicted to be active, providing insight into its function and cellular context.
  • Cytoplasm
  • Mitochondria
  • cytosol
  • mitochondrial matrix
  • mitochondrial inner membrane

Gene Ontology Annotations

Describes the biological processes, cellular components, and molecular functions associated with the human COQ3 gene, providing context for its role in the cell.

Biological Process

Functions and activities the gene product is involved in
  • glycerol metabolic process
  • ubiquinone biosynthetic process via 3,4-dihydroxy-5-polyprenylbenzoate
  • ubiquinone biosynthetic process
  • methylation

Cellular Component

Where in the cell the gene product is active
  • mitochondrial matrix
  • mitochondrion
  • extrinsic to mitochondrial inner membrane
  • mitochondrial inner membrane

Molecular Function

What the gene product does at the molecular level
  • O-methyltransferase activity
  • protein binding
  • polyprenyldihydroxybenzoate methyltransferase activity
  • 2-polyprenyl-6-methoxy-1,4-benzoquinone methyltransferase activity

Gene-Specific Assays for Results You Can Trust

Streamline your workflow with assays designed for this gene. Our targeted dPCR and qPCR assays help you generate meaningful data – efficiently and accurately.