摘要
核黄素或维生素B2,在细胞中作为FAD和FMN生物前体有重要作用,为黄素蛋白的结构和功能必不可少的两个重要的黄素辅因子。核黄素已应用于先天性代谢的各种治疗方法,尤其是在代谢紊乱导致的无论是从参与了核黄素代谢和运输或缺陷在黄素酶蛋白缺陷。本次总结的范围是提供一个更新的角度来看,在临床情况下,使用的是作为一个潜在的治疗剂在影响线粒体能量代谢紊乱的临床情况下。特别是,我们讨论了解核黄素作为错误折叠的代谢黄素酶复苏药物分子伴侣的作用的机理。
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
图形摘要
Current Drug Targets
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.
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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
DOI https://dx.doi.org/10.2174/1389450117666160813180812 |
Print ISSN 1389-4501 |
Publisher Name Bentham Science Publisher |
Online ISSN 1873-5592 |
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