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如何治疗线粒体心肌病中的“心上人”。

How to treat a "sweetheart" in mitochondrial cardiomyopathy.

机构信息

Inherited Movement Disorders Unit, Neurogenetics Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD, 20892, USA.

出版信息

EMBO Mol Med. 2024 Jun;16(6):1226-1227. doi: 10.1038/s44321-024-00070-w. Epub 2024 May 9.

Abstract

Mitochondria are the powerhouses of the cell, and they are fuel flexible. They can generate power from nearly every abundant carbon source in the body, including carbohydrates, amino acids, and fatty acids. When mitochondria are sick, however, some fuels may be better than others – and the best may not come in “heart healthy” packaging. In this issue of EMBO Molecular Medicine, Southwell et al, report dramatic rescue of a mitochondrial cardiomyopathy by forcing sick mitochondria to use fatty acids, delivered through a high fat diet, rather than sweet glucose they seem to prefer.

摘要

线粒体是细胞的动力源,它们的燃料具有多样性。它们可以利用体内几乎所有丰富的碳源来产生能量,包括碳水化合物、氨基酸和脂肪酸。然而,当线粒体出现问题时,某些燃料可能比其他燃料更好——而最好的燃料可能并不来自“心脏健康”的包装。在本期的《EMBO 分子医学》中,Southwell 等人报告称,通过强制患病的线粒体使用高脂肪饮食中提供的脂肪酸,而不是它们似乎更喜欢的甜味葡萄糖,可显著挽救一种线粒体心肌病。

相似文献

1
How to treat a "sweetheart" in mitochondrial cardiomyopathy.如何治疗线粒体心肌病中的“心上人”。
EMBO Mol Med. 2024 Jun;16(6):1226-1227. doi: 10.1038/s44321-024-00070-w. Epub 2024 May 9.
4
Risks inherent to mitochondrial replacement.线粒体替代所固有的风险。
EMBO Rep. 2015 May;16(5):541-4. doi: 10.15252/embr.201439110. Epub 2015 Mar 25.
7
Progress in Molecular Genetic Study of Mitochondrial Cardiomyopathy.线粒体心肌病的分子遗传学研究进展
Zhongguo Yi Xue Ke Xue Yuan Xue Bao. 2017 Jun 20;39(3):438-444. doi: 10.3881/j.issn.1000-503X.2017.03.024.

本文引用的文献

8
Ketogenic diet slows down mitochondrial myopathy progression in mice.生酮饮食可减缓小鼠线粒体肌病的进展。
Hum Mol Genet. 2010 May 15;19(10):1974-84. doi: 10.1093/hmg/ddq076. Epub 2010 Feb 17.

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