Mitochondrial LonP1 is an essential stress response protease that mediates mitochondrial proteostasis, metabolism and bioenergetics. Homozygous and compound heterozygous variants in the LONP1 gene encoding the LonP1 protease have recently been shown to cause a diverse spectrum of human pathologies, ranging from classical mitochondrial disease phenotypes, in addition to profound neurologic impairment and multi-organ dysfunctions, some of which are uncommon to mitochondrial disorders. In this review, we focus primarily on human LonP1 and discuss findings, which demonstrate its multidimensional roles in maintaining mitochondrial proteostasis and adapting cells to metabolic flux and stress during normal physiology and disease processes. We also discuss emerging roles of LonP1 in responding to developmental, oncogenic and cardiac stress.

中文翻译：

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线粒体 LonP1 蛋白酶对细胞压力的管理——减轻发育、致癌、心脏压力

线粒体 LonP1 是一种必不可少的应激反应蛋白酶，可介导线粒体蛋白质稳态、代谢和生物能学。编码 LonP1 蛋白酶的 LONP1 基因中的纯合和复合杂合变体最近已被证明会导致多种人类病理，包括经典的线粒体疾病表型，以及严重的神经功能障碍和多器官功能障碍，其中一些不常见到线粒体疾病。在这篇综述中，我们主要关注人类 LonP1 并讨论发现，这些发现证明了它在维持线粒体蛋白质稳态和使细胞适应正常生理和疾病过程中的代谢通量和压力方面的多维作用。我们还讨论了 LonP1 在应对发育、致癌和心脏压力方面的新作用。