ABSTRACT Caseinolytic protease (CLPP) is an energy-dependent serine-type protease that plays a role in protein quality control. The CLPP gene is highly conserved across kingdoms and the protein is present in both bacteria and eukaryote organelles like mitochondria across a wide phylogenetic range. This pedigree has all the hallmarks of CLPP being an essential gene. However, in plants, disruption of mitochondrial CLPP has no impact on its growth, reminiscent of its nonessential role in some model fungi. Deletion of mitochondrial CLPP improves health and increased life span in the filamentous fungus, Podospora anserina, while loss of human mitochondrial CLPP leads to infertility and hearing loss. Recently it was revealed that both plant and human CLPP share a similar role in maintenance of the N-module of respiratory complex I. In addition, plant mitochondrial CLPP also coordinates the homeostasis of other mitochondrial protein complexes encoded by genes across mitochondrial and nuclear genomes. Understanding the contextual role of mitochondrial CLPP across kingdoms may help to understand these diverse sets of clpp phenotypes and the widespread conservation of CLPP genes.

中文翻译：

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保守的线粒体 CLPP 及其功能的丧失导致植物和其他生物体的不同表型

摘要酪蛋白水解蛋白酶 (CLPP) 是一种能量依赖性丝氨酸型蛋白酶，在蛋白质质量控​​制中发挥作用。CLPP 基因在跨界高度保守，并且该蛋白质存在于细菌和真核生物细胞器（如线粒体）的广泛系统发育范围内。该谱系具有 CLPP 作为必需基因的所有特征。然而，在植物中，线粒体 CLPP 的破坏对其生长没有影响，让人想起它在某些模型真菌中的非必要作用。线粒体 CLPP 的缺失可改善丝状真菌 Podospora anserina 的健康状况并延长其寿命，而人类线粒体 CLPP 的缺失会导致不育和听力损失。最近发现植物和人类 CLPP 在维持呼吸复合体 I 的 N 模块方面具有相似的作用。 此外，植物线粒体 CLPP 还协调由跨线粒体和核基因组的基因编码的其他线粒体蛋白复合物的稳态。了解线粒体 CLPP 跨界的上下文作用可能有助于了解这些不同的 clpp 表型集和 CLPP 基因的广泛保护。