Oxidative stress, bioenergetics and neurodegenerative diseases

Abstract

Neurodegenerative diseases (NDD) are an assortment of clinical central nervous system (CNS) disorders, characterized by intraneuronal accumulation of fibrillary material, forming intracellular aggregates. These abnormal products play a prominent role in the dysfunction and neuronal destruction that is specific for NDD, such as Alzheimer's, Parkinson's, and prion‐related diseases, known as transmissible spongiform encephalopathies. Related to this group are some neurological syndromes that involve muscle and CNS; Huntington's chorea, Friedreich's ataxia, and amyotrophic lateral sclerosis. A number of mechanisms appear to initiate or contribute to the neurodegenerative process, including oxidative stress, resulting from free radical generation and mitochondrial dysfunction. Overall, the clinical data on antioxidant efficacy tend to be encouraging. Moreover, the brain is highly dependent on energy availability, and mitochondrial functional impairment results in tissue injury, with detrimental clinical manifestations. Coenzyme Q (coenzyme Q10 in humans) is an essential component of the electron tranport chain in the mitochondria and the intracellular energy generation. It is also a potent antioxidant and a membrane stabilizer. For this reason, the use of coenzyme Q10 (and its derivative idebenone) in the prevention and treatment of NDD is fundamentally justified. Anecdotal, as well as preliminary results from well‐designed long‐term coenzyme Q10 clinical trials reinforce the validity of this novel approach. Due to the aging population, NDD have become a major health and social problem, and conceiving treatment or even arresting progression has become a primary focus of medical research worldwide.