在线粒体能量代谢紊乱中使用核黄素的治疗方法

Title:Therapeutic Approaches Using Riboflavin in Mitochondrial Energy Metabolism Disorders

Volume: 17 Issue: 13

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

Affiliation:

关键词: 核黄素反应性，黄素酶，核黄素转运、代谢紊乱，线粒体β氧化障碍，呼吸链缺陷

摘要: 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:

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