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Updated: Jul 12, 2025

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在复杂I缺乏症期间,α-甲酸脱酶的功能缺陷加剧了线粒体ATP缺陷

Gerardo G Piroli1, Allison M Manuel1, Richard S McCain1

  • 1Department of Pharmacology, Physiology & Neuroscience, School of Medicine, University of South Carolina, Columbia, SC, 29209, USA.

Redox biology
|October 26, 2023
PubMed
概括

在NDUFS4淘汰赛小鼠中,线粒体复合体I缺陷导致蛋白质吞,损害TCA循环并导致神经退行. 这项研究揭示了在利氏综合征模型中除了OXPHOS功能障碍之外的新型代谢缺陷.

关键词:
阿尔法-甲酸盐脱酶.综合体I 是一个综合体.烟酸的使用方法李氏综合征 (Leigh综合征) 是一种蛋白质的顺化 蛋白质的顺在基质水平上进行酸化.

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