线粒体疾病中的高代谢和能量限制
Hypermetabolism and energetic constraints in mitochondrial disorders.
作者信息
Sercel Alexander J, Sturm Gabriel, Gallagher Dympna, St-Onge Marie-Pierre, Kempes Christopher P, Pontzer Herman, Hirano Michio, Picard Martin
机构信息
Department of Psychiatry, Division of Behavioral Medicine, Columbia University Irving Medical Center, New York, USA.
Department of Biochemistry and Biophysics, University of California, San Francisco, California, USA.
出版信息
Nat Metab. 2024 Feb;6(2):192-195. doi: 10.1038/s42255-023-00968-8.
Abstract
The prevailing notion that mitochondrial diseases arise from ATP deficiency is challenged by recent evidence that oxidative phosphorylation defects trigger maladaptive stress responses consuming excess energy. We argue that this chronic state of hypermetabolism imposes energetic constraints, thus causing mitochondrial disease pathophysiology, calling for careful translational studies from organelle to organism.
摘要
线粒体疾病源于ATP缺乏这一普遍观念受到了近期证据的挑战，该证据表明氧化磷酸化缺陷会引发消耗过多能量的适应性应激反应。我们认为，这种慢性高代谢状态会带来能量限制，从而导致线粒体疾病的病理生理过程，这需要从细胞器到生物体进行细致的转化研究。
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