Bypassing human CoQ10 deficiency

Mol Genet Metab. 2018 Mar;123(3):289-291. doi: 10.1016/j.ymgme.2017.12.008. Epub 2017 Dec 9.

Abstract

Primary disorders of the human coenzyme Q10 (CoQ10) biosynthesis pathway are a known cause of severe pediatric diseases. So far, oral administration of CoQ10 is the only treatment strategy for affected individuals. However, the real benefit of CoQ10 supplementation remains questionable and clinical studies regarding efficiency are lacking. Here we provide an outlook on novel treatment approaches using CoQ precursor compounds. These metabolic bypass strategies might be a promising alternative for oral CoQ10 supplementation regimens.

Keywords: Coenzyme Q(10); Kidney; Multiple system atrophy; Neurological; Treatment.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Apoptosis / drug effects
  • Ataxia / drug therapy*
  • Ataxia / genetics
  • Ataxia / pathology
  • Biosynthetic Pathways / drug effects
  • Biosynthetic Pathways / genetics
  • Cytochrome P-450 Enzyme System / genetics
  • Cytochrome P-450 Enzyme System / metabolism
  • Disease Models, Animal
  • Humans
  • Hydroxybenzoates / pharmacology
  • Hydroxybenzoates / therapeutic use*
  • Mice
  • Mitochondria / metabolism
  • Mitochondrial Diseases / drug therapy*
  • Mitochondrial Diseases / genetics
  • Mitochondrial Diseases / pathology
  • Muscle Weakness / drug therapy*
  • Muscle Weakness / genetics
  • Muscle Weakness / pathology
  • Pyrimidines / metabolism
  • Solubility
  • Treatment Outcome
  • Ubiquinone / analogs & derivatives*
  • Ubiquinone / biosynthesis
  • Ubiquinone / deficiency*
  • Ubiquinone / genetics
  • Ubiquinone / metabolism
  • Ubiquinone / therapeutic use
  • Vitamins / therapeutic use

Substances

  • Hydroxybenzoates
  • Pyrimidines
  • Vitamins
  • Ubiquinone
  • Cytochrome P-450 Enzyme System
  • coenzyme Q10
  • Ubiquinone Q2
  • pyrimidine

Supplementary concepts

  • Coenzyme Q10 Deficiency