Para-hydroxybenzoate—polyprenyltransferase, mitochondrial is an enzyme that in humans is encoded by the COQ2 gene. [5] [6] CoQ serves as a redox carrier in the mitochondrial respiratory chain and is a lipid-soluble antioxidant.

Dec 24, 2024 · COQ2 (Coenzyme Q2, Polyprenyltransferase) is a Protein Coding gene. Diseases associated with COQ2 include Multiple System Atrophy 1 and Coenzyme Q10 Deficiency, Primary, 1. Among its related pathways are Peroxisomal lipid metabolism and Metabolism of water-soluble vitamins and cofactors.

The COQ2 gene provides instructions for making an enzyme that carries out one step in the production of a molecule called coenzyme Q10, which has several critical functions in cells throughout the body. Learn about this gene and related health conditions.

Jan 19, 2023 · Coenzyme Q (or ubiquinone) is a redox-active lipid that serves as universal electron carrier in the mitochondrial respiratory chain and antioxidant in the plasma membrane limiting lipid...

Oct 23, 2023 · CoQ (ubiquinone) serves as a redox carrier in the mitochondrial respiratory chain and is a lipid-soluble antioxidant. COQ2, or parahydroxybenzoate-polyprenyltransferase (EC 2.5.1.39), catalyzes one of the final reactions in the biosynthesis of CoQ, the prenylation of parahydroxybenzoate with an all-trans polyprenyl group (Forsgren et al., 2004).

Gene ID: 27235, updated on 8-Feb-2025. This gene encodes an enzyme that functions in the final steps in the biosynthesis of CoQ (ubiquinone), a redox carrier in the mitochondrial respiratory chain and a lipid-soluble antioxidant.

Jul 18, 2013 · We identified a homozygous mutation (M128V-V393A/M128V-V393A) and compound heterozygous mutations (R387X/V393A) in COQ2 in two multiplex families. Furthermore, we found that a common variant...

Title: Identification of COQ2 as a regulator of proliferation and lipid peroxidation through genome-scale CRISPR-Cas9 screening in myeloma cells. COQ2 and SNCA polymorphisms interact with environmental factors to modulate the risk of multiple system atrophy and subtype disposition.

Oct 1, 2016 · COQ2 (p-hydroxybenzoate polyprenyl transferase) encodes the enzyme required for the second step of the final reaction sequence of Coenzyme Q 10 (CoQ) biosynthesis. Its mutations represent a frequent cause of primary CoQ deficiency and have been associated with the widest clinical spectrum, …

Sep 19, 2024 · Clinical resource with information about COQ2, Coenzyme Q10 deficiency, primary, 1, Multiple system atrophy 1, susceptibility to, and available tests. There are links to practice guidelines and authoritative resources like GeneReviews, PubMed, MedlinePlus, PharmGKB and clinicaltrials.gov.