Therapeutic Approaches Using Riboflavin in Mitochondrial Energy Metabolism Disorders

Title:Therapeutic Approaches Using Riboflavin in Mitochondrial Energy Metabolism Disorders

Volume: 17 Issue: 13

Author(s): Bárbara J. Henriques, Tânia G. Lucas and Cláudio M. Gomes

Affiliation:

Keywords: Riboflavin-responsive, flavoenzymes, riboflavin transporters, metabolic disorders, mitochondrial beta-oxidation disorders, respiratory chain deficiencies.

Abstract: Riboflavin, or vitamin B2, plays an important role in the cell as biological precursor of FAD and FMN, two important flavin cofactors which are essential for the structure and function of flavoproteins. Riboflavin has been used in therapeutic approaches of various inborn errors of metabolism, notably in metabolic disorders resulting either from defects in proteins involved in riboflavin metabolism and transport or from defects in flavoenzymes. The scope of this review is to provide an updated perspective of clinical cases in which riboflavin was used as a potential therapeutic agent in disorders affecting mitochondrial energy metabolism. In particular, we discuss available mechanistic insights on the role of riboflavin as a pharmacological chaperone for the recovery of misfolded metabolic flavoenzymes.

Cite this article as:

Henriques J. Bárbara, Lucas G. Tânia and Gomes M. Cláudio, Therapeutic Approaches Using Riboflavin in Mitochondrial Energy Metabolism Disorders, Current Drug Targets 2016; 17 (13) . https://dx.doi.org/10.2174/1389450117666160813180812