COQ2 : coenzyme Q2, polyprenyltransferase
Description
The COQ2 (coenzyme Q2, polyprenyltransferase) is a protein-coding gene located on chromosome 4.
The COQ2 gene is responsible for producing an enzyme that plays a crucial role in the production of coenzyme Q10 (CoQ10), a vital molecule with several essential functions in the body. In mitochondria, CoQ10 is involved in energy production, helping to convert food into a form cells can use. It also participates in the synthesis of pyrimidines, which are building blocks for DNA and RNA, as well as energy sources in the cell. Additionally, CoQ10 acts as an antioxidant in cell membranes, protecting them from damage caused by free radicals, which are byproducts of energy production.
The COQ2 gene provides instructions for making an enzyme called 4-hydroxybenzoate polyprenyltransferase, which plays a key role in the production of coenzyme Q10 (CoQ10). This enzyme catalyzes the addition of a long chain of isoprene units (decaprenyl) to a molecule called para-hydroxybenzoate (PHB), forming the precursor to CoQ10. This prenylation reaction is a crucial step in the final biosynthesis of CoQ10. The length of the prenyl chain varies between species, with humans producing CoQ10, while rodents primarily produce CoQ9. However, there is some overlap in the production of these two forms. CoQ10 is vital for cellular energy production, as it acts as an electron carrier in the mitochondrial respiratory chain, helping to convert food into usable energy. Additionally, CoQ10 plays a role in regulating cell death, specifically in controlling the opening of mitochondrial permeability transition pores and the production of reactive oxygen species (ROS).
COQ2 is also known as CL640, COQ10D1, MSA1, PHB:PPT.
Associated Diseases
- Multiple system atrophy, parkinsonian type
- Multiple system atrophy 1, susceptibility to
- Coenzyme Q10 deficiency, primary, 1
- Multiple system atrophy, cerebellar type
- Multiple system atrophy
- Primary coenzyme Q10 deficiency
