Document type
Journal articles
Document subtype
Full paper
Title
Therapeutic Approaches Using Riboflavin in Mitochondrial Energy Metabolism Disorders
Participants in the publication
Bárbara J. Henriques (Author)
Dep. Química e Bioquímica
BioISI
Tânia G. Lucas (Author)
Cláudio M. Gomes (Author)
Dep. Química e Bioquímica
BioISI
Summary
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.
Date of Publication
2016-09-02
Institution
Biosystems & Integrative Sciences Institute, Faculdade de Ciências, Universidade de Lisboa, Portugal.
Where published
Current Drug Targets
Publication Identifiers
ISSN - 1389-4501
Publisher
Bentham Science Publishers Ltd.
Number of pages
7
Starting page
1527
Last page
1534
Document Identifiers
URL -
http://dx.doi.org/10.2174/1389450117666160813180812
DOI -
https://doi.org/10.2174/1389450117666160813180812
Rankings
Web Of Science Q2 (2017) - 3.112 - PHARMACOLOGY & PHARMACY - SCIE
Web Of Science Q2 (2018) - 2.642 - PHARMACOLOGY & PHARMACY - SCIE
Keywords
Riboflavin-responsive
flavoenzymes
riboflavin transporters
metabolic disorders
mitochondrial beta-oxidation disorders
respiratory chain deficiencies