Inherited Movement Disorders Unit, Neurogenetics Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, MD, 20892, USA.
EMBO Mol Med. 2024 Jun;16(6):1226-1227. doi: 10.1038/s44321-024-00070-w. Epub 2024 May 9.
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.
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。
