title: Therapeutic Approaches Using Riboflavin in Mitochondrial Energy Metabolism Disorders.
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.
authors.forename: Bárbara J
authors.initials: BJ
authors.lastname: Henriques
authors.forename: Tânia G
authors.initials: TG
authors.lastname: Lucas
authors.affiliation: Faculdade de Ciências Universidade de Lisboa, Biosystems and Integrative Sciences Institute and Department of Chemistry and Biochemistry, Universidade de Lisboa, Campo Grande, 1749-016 Lisboa, Portugal. cmgomes@fc.ul.pt.
authors.forename: Cláudio M
authors.initials: CM
authors.lastname: Gomes
mesh_terms: D004734:Energy Metabolism / Q000187:drug effects*; D006801:Humans; D008928:Mitochondria / Q000378:metabolism; D028361:Mitochondrial Diseases / Q000188:drug therapy* / Q000503:physiopathology; D017510:Protein Folding; D012256:Riboflavin / Q000378:metabolism / Q000494:pharmacology / Q000627:therapeutic use*; D014803:Vitamin B Complex / Q000378:metabolism / Q000494:pharmacology / Q000627:therapeutic use
chemical_list: D014803:Vitamin B Complex; D012256:Riboflavin