Cellular Consequences of Coenzyme Q10 Deficiency in Neurodegeneration of the Retina and Brain

Int J Mol Sci. 2020 Dec 6;21(23):9299. doi: 10.3390/ijms21239299.

Abstract

Coenzyme Q10 (CoQ10) is a ubiquitous cofactor in the body, operating in the inner mitochondrial membrane, where it plays a vital role in the generation of adenosine triphosphate (ATP) through the electron transport chain (ETC). In addition to this, CoQ10 serves as an antioxidant, protecting the cell from oxidative stress by reactive oxygen species (ROS) as well as maintaining a proton (H+) gradient across lysosome membranes to facilitate the breakdown of cellular waste products. Through the process of ageing, the body becomes deficient in CoQ10, resulting in several systemic manifestations. On a cellular level, one of the consequences of CoQ10 deficiency is apoptosis, which can be visualised in tissues of the central nervous system (CNS). Diseases affecting the retina and brain such as age-related macular degeneration (AMD), glaucoma, Alzheimer's disease (AD) and Parkinson's disease (PD) have shown defects in cellular biochemical reactions attributed to reduced levels of CoQ10. Through further research into the pathogenesis of such conditions, the effects of CoQ10 deficiency can be counteracted through supplementation, early detection and intervention.

Keywords: apoptosis; coenzyme Q10; neurodegeneration; reactive oxygen species.

Publication types

  • Review

MeSH terms

  • Animals
  • Ataxia / complications
  • Ataxia / metabolism*
  • Ataxia / pathology
  • Brain / metabolism*
  • Brain / pathology
  • Humans
  • Mitochondrial Diseases / complications
  • Mitochondrial Diseases / metabolism*
  • Mitochondrial Diseases / pathology
  • Muscle Weakness / complications
  • Muscle Weakness / metabolism*
  • Muscle Weakness / pathology
  • Neurodegenerative Diseases / etiology
  • Neurodegenerative Diseases / metabolism*
  • Neurodegenerative Diseases / pathology
  • Retina / metabolism*
  • Retina / pathology
  • Ubiquinone / analogs & derivatives
  • Ubiquinone / deficiency*
  • Ubiquinone / metabolism

Substances

  • Ubiquinone
  • coenzyme Q10

Supplementary concepts

  • Coenzyme Q10 Deficiency