Gene COQ2

Also known as

CL640, PHB:polyprenyltransferase, coenzyme Q2

Overview

COQ2 (Coenzyme Q2 4-Hydroxybenzoate Polyprenyltransferase) encodes an enzyme essential for the biosynthesis of coenzyme Q10 (ubiquinone). This enzyme catalyzes the prenylation step in CoQ10 synthesis, attaching the isoprenoid side chain to the benzoquinone ring. CoQ10 is critical for mitochondrial electron transport and serves as a powerful antioxidant.

Mutations in COQ2 cause primary CoQ10 deficiency, a mitochondrial disorder with variable presentations including infantile multisystem disease, nephropathy with sensorineural deafness, and cerebellar ataxia. CoQ10 supplementation can be life-saving if started early. Common variants in COQ2 have also been associated with Parkinson's disease and multiple system atrophy in some studies, suggesting a role in neurodegenerative disorders.

Understanding your COQ2 genetic status provides insights into CoQ10 synthesis capacity and mitochondrial function. This information may be relevant for understanding response to CoQ10 supplementation and susceptibility to mitochondrial dysfunction.

NutraHacker Raw DNA Analysis Products that include this gene

• Carrier Status and Drug Response Report

Upload raw DNA data to get your very own analysis of gene COQ2 through your personalized DNA reports.