Suppr超能文献

核黄素缺乏症——对人类整体健康和先天性代谢错误的影响。

Riboflavin Deficiency-Implications for General Human Health and Inborn Errors of Metabolism.

机构信息

Research Unit for Molecular Medicine, Department for Clinical Medicine, Aarhus University and Aarhus University Hospital, 8200 Aarhus N, Denmark.

Department of Biosciences, Biotechnology and Biopharmaceutics, University of Bari 'Aldo Moro', 70121 Bari, Italy.

出版信息

Int J Mol Sci. 2020 May 28;21(11):3847. doi: 10.3390/ijms21113847.

Abstract

As an essential vitamin, the role of riboflavin in human diet and health is increasingly being highlighted. Insufficient dietary intake of riboflavin is often reported in nutritional surveys and population studies, even in non-developing countries with abundant sources of riboflavin-rich dietary products. A latent subclinical riboflavin deficiency can result in a significant clinical phenotype when combined with inborn genetic disturbances or environmental and physiological factors like infections, exercise, diet, aging and pregnancy. Riboflavin, and more importantly its derivatives, flavin mononucleotide (FMN) and flavin adenine dinucleotide (FAD), play a crucial role in essential cellular processes including mitochondrial energy metabolism, stress responses, vitamin and cofactor biogenesis, where they function as cofactors to ensure the catalytic activity and folding/stability of flavoenzymes. Numerous inborn errors of flavin metabolism and flavoenzyme function have been described, and supplementation with riboflavin has in many cases been shown to be lifesaving or to mitigate symptoms. This review discusses the environmental, physiological and genetic factors that affect cellular riboflavin status. We describe the crucial role of riboflavin for general human health, and the clear benefits of riboflavin treatment in patients with inborn errors of metabolism.

摘要

作为一种必需维生素,核黄素在人类饮食和健康中的作用越来越受到重视。营养调查和人群研究经常报告膳食中核黄素摄入不足,即使在核黄素丰富的饮食产品来源丰富的非发展中国家也是如此。当与先天遗传紊乱或感染、运动、饮食、衰老和妊娠等环境和生理因素结合时,潜在的亚临床核黄素缺乏会导致显著的临床表型。核黄素及其更重要的衍生物黄素单核苷酸(FMN)和黄素腺嘌呤二核苷酸(FAD)在包括线粒体能量代谢、应激反应、维生素和辅助因子生物发生在内的基本细胞过程中发挥着关键作用,它们作为辅助因子确保黄素酶的催化活性和折叠/稳定性。已经描述了许多黄素代谢和黄素酶功能的先天错误,并且在许多情况下,补充核黄素已被证明可以挽救生命或减轻症状。本文讨论了影响细胞核黄素状态的环境、生理和遗传因素。我们描述了核黄素对人类健康的重要性,以及核黄素治疗代谢先天错误患者的明显益处。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0e22/7312377/a3267419fa2d/ijms-21-03847-g001.jpg

文献AI研究员

20分钟写一篇综述,助力文献阅读效率提升50倍。

立即体验

用中文搜PubMed

大模型驱动的PubMed中文搜索引擎

马上搜索

文档翻译

学术文献翻译模型,支持多种主流文档格式。

立即体验