心功能不全导致的辅酶A平衡受损以及用维生素B5及其衍生物促进辅酶A生成对治疗心力衰竭的益处

IF 4.2 2区 医学 Q1 ENDOCRINOLOGY & METABOLISM
J. J. Wedman, O. C. M. Sibon, E. Mastantuono, A. Iuso
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引用次数: 0

摘要

辅酶 A(CoA)是人体内百余种代谢反应所必需的重要辅因子。这种辅因子在细胞中由维生素 B5(又称泛酸)从头合成,维生素 B5 是一种水溶性维生素,大量存在于蔬菜和动物性食物中。神经退行性疾病、癌症和传染病都与 CoA 从头生物合成的缺陷或这种辅酶水平的降低有关。现在有越来越多的证据表明,CoA 的限制也是心脏功能障碍的一个关键病理机制。在本综述中,我们将总结目前有关 CoA 和心力衰竭的知识,重点介绍两种主要的心肌病--磷酸泛硫酰半胱氨酸合成酶和磷酸泛硫酰半胱氨酸脱羧酶缺乏症,这两种疾病的生化特征是患者样本中的 CoA 水平降低。因此,我们将讨论维生素 B5 及其衍生物泛硫乙氨酸和 4′-磷泛硫乙氨酸恢复 CoA 对这些疾病的潜在益处,更广泛地说,是对心力衰竭的潜在益处。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Impaired coenzyme A homeostasis in cardiac dysfunction and benefits of boosting coenzyme A production with vitamin B5 and its derivatives in the management of heart failure

Impaired coenzyme A homeostasis in cardiac dysfunction and benefits of boosting coenzyme A production with vitamin B5 and its derivatives in the management of heart failure

Coenzyme A (CoA) is an essential cofactor required for over a hundred metabolic reactions in the human body. This cofactor is synthesized de novo in our cells from vitamin B5, also known as pantothenic acid, a water-soluble vitamin abundantly present in vegetables and animal-based foods. Neurodegenerative disorders, cancer, and infectious diseases have been linked to defects in de novo CoA biosynthesis or reduced levels of this coenzyme. There is now accumulating evidence that CoA limitation is a critical pathomechanism in cardiac dysfunction too. In the current review, we will summarize our current knowledge on CoA and heart failure, with emphasis on two primary cardiomyopathies, phosphopantothenoylcysteine synthetase and phosphopantothenoylcysteine decarboxylase deficiency disorders biochemically characterized by a decreased level of CoA in patients' samples. Hence, we will discuss the potential benefits of CoA restoration in these diseases and, more generally, in heart failure, by vitamin B5 and its derivatives pantethine and 4′-phosphopantetheine.

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来源期刊
Journal of Inherited Metabolic Disease
Journal of Inherited Metabolic Disease 医学-内分泌学与代谢
CiteScore
9.50
自引率
7.10%
发文量
117
审稿时长
4-8 weeks
期刊介绍: The Journal of Inherited Metabolic Disease (JIMD) is the official journal of the Society for the Study of Inborn Errors of Metabolism (SSIEM). By enhancing communication between workers in the field throughout the world, the JIMD aims to improve the management and understanding of inherited metabolic disorders. It publishes results of original research and new or important observations pertaining to any aspect of inherited metabolic disease in humans and higher animals. This includes clinical (medical, dental and veterinary), biochemical, genetic (including cytogenetic, molecular and population genetic), experimental (including cell biological), methodological, theoretical, epidemiological, ethical and counselling aspects. The JIMD also reviews important new developments or controversial issues relating to metabolic disorders and publishes reviews and short reports arising from the Society''s annual symposia. A distinction is made between peer-reviewed scientific material that is selected because of its significance for other professionals in the field and non-peer- reviewed material that aims to be important, controversial, interesting or entertaining (“Extras”).
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