COQ2/YNR041C Summary Help

Standard Name COQ2
Systematic Name YNR041C
Feature Type ORF, Verified
Description Para hydroxybenzoate polyprenyl transferase; catalyzes the second step in ubiquinone (coenzyme Q) biosynthesis; human COQ2, mutations in which are implicated in an increased risk of mutiple-system atrophy, is able to complement a yeast coq2 null mutant (1 and see Summary Paragraph)
Name Description COenzyme Q
Chromosomal Location
ChrXIV:701659 to 700541 | ORF Map | GBrowse
Note: this feature is encoded on the Crick strand.
Gene Ontology Annotations All COQ2 GO evidence and references
  View Computational GO annotations for COQ2
Molecular Function
Manually curated
Biological Process
Manually curated
Cellular Component
Manually curated
Regulators 6 genes
Classical genetics
reduction of function
Large-scale survey
142 total interaction(s) for 115 unique genes/features.
Physical Interactions
  • Affinity Capture-MS: 1
  • Affinity Capture-RNA: 1
  • PCA: 2
  • Reconstituted Complex: 1
  • Two-hybrid: 2

Genetic Interactions
  • Dosage Rescue: 1
  • Negative Genetic: 95
  • Positive Genetic: 18
  • Synthetic Growth Defect: 19
  • Synthetic Lethality: 2

Expression Summary
Length (a.a.) 372
Molecular Weight (Da) 41,001
Isoelectric Point (pI) 10.81
Phosphorylation PhosphoGRID | PhosphoPep Database
sequence information
ChrXIV:701659 to 700541 | ORF Map | GBrowse
Note: this feature is encoded on the Crick strand.
Last Update Coordinates: 2011-02-03 | Sequence: 1997-01-28
Subfeature details
Most Recent Updates
Coordinates Sequence
CDS 1..1119 701659..700541 2011-02-03 1997-01-28
Retrieve sequences
Analyze Sequence
S288C only
S288C vs. other species
S288C vs. other strains
External Links All Associated Seq | E.C. | Entrez Gene | Entrez RefSeq Protein | MIPS | Search all NCBI (Entrez) | UniProtKB
Primary SGDIDS000005324

COQ2 encodes para-hydroxybenzoate:polyprenyltransferase, an enzyme of the ubiquinone (Coenzyme Q) biosynthetic pathway (1). Ubiquinone serves to transport electrons between the respiratory enzyme complexes of the mitochondrial inner membrane. Coq2p catalyzes the prenylation of para-hydroxybenzoate to form 3-hexaprenyl-4-hydroxybenzoate, which is the second committed step in the pathway. coq2 mutants display a ubiquinone biosynthetic defect, lack para-hydroxybenzoate:polyprenyltransferase activity, and also exhibit a respiratory growth defect (inability to grow on nonfermentable carbon sources) (1).

Coq2p is located in the mitochondrion (2, 1, 3) and is likely to be a constituent of the inner membrane, as is its rat ortholog (4). Genetic interactions suggest that the ubiquinone biosynthetic enzymes Coq1p, Coq2p, Coq3p, Coq4p, Coq5p, Coq6p, Cat5p, and Coq8p may form a complex in the mitochondrion (5, 6). A substantial amount of Coq2p activity also copurifies with the microsomal fraction, but the significance of this is unclear (1).

COQ2 is well-conserved, with cross-complementing orthologs found in species from bacteria (7) to mammals (8) and plants (9). Mutations in the human ortholog COQ2 (OMIM) cause primary Coenzyme Q10 deficiency (10).

Last updated: 2006-09-13 Contact SGD

References cited on this page View Complete Literature Guide for COQ2
1) Ashby MN, et al.  (1992) COQ2 is a candidate for the structural gene encoding para-hydroxybenzoate:polyprenyltransferase. J Biol Chem 267(6):4128-36
2) Casey J and Threlfall DR  (1978) Formation of 3-hexaprenyl-4-hydroxybenzoate by matrix-free mitochondrial membrane-rich preparations of yeast. Biochim Biophys Acta 530(3):487-502
3) Leuenberger D, et al.  (1999) Different import pathways through the mitochondrial intermembrane space for inner membrane proteins. EMBO J 18(17):4816-22
4) Kang D, et al.  (1992) Ubiquinone biosynthesis by mitochondria, sonicated mitochondria, and mitoplasts of rat liver. J Biochem (Tokyo) 111(3):371-5
5) Hsu AY, et al.  (2000) Genetic evidence for a multi-subunit complex in the O-methyltransferase steps of coenzyme Q biosynthesis. Biochim Biophys Acta 1484(2-3):287-97
6) Gin P and Clarke CF  (2005) Genetic evidence for a multi-subunit complex in coenzyme Q biosynthesis in yeast and the role of the Coq1 hexaprenyl diphosphate synthase. J Biol Chem 280(4):2676-81
7) Suzuki K, et al.  (1994) Evidence that Escherichia coli ubiA product is a functional homolog of yeast COQ2, and the regulation of ubiA gene expression. Biosci Biotechnol Biochem 58(10):1814-9
8) Forsgren M, et al.  (2004) Isolation and functional expression of human COQ2, a gene encoding a polyprenyl transferase involved in the synthesis of CoQ. Biochem J 382(Pt 2):519-26
9) Okada K, et al.  (2004) The AtPPT1 gene encoding 4-hydroxybenzoate polyprenyl diphosphate transferase in ubiquinone biosynthesis is required for embryo development in Arabidopsis thaliana. Plant Mol Biol 55(4):567-77
10) Quinzii C, et al.  (2006) A mutation in para-hydroxybenzoate-polyprenyl transferase (COQ2) causes primary coenzyme Q10 deficiency. Am J Hum Genet 78(2):345-9