Organismal and Cellular Stress Responses upon Disruption of Mitochondrial Lonp1 Protease.

ABSTRACT: Cells engage complex surveillance mechanisms to maintain mitochondrial function and protein homeostasis. LonP1 protease is a key component of mitochondrial quality control and has been implicated in human malignancies and other pathological disorders. Here, we employed two experimental systems, the worm Caenorhabditis elegans and human cancer cells, to investigate and compare the effects of LONP-1/LonP1 deficiency at the molecular, cellular, and organismal levels. Deletion of the lonp-1 gene in worms disturbed mitochondrial function, provoked reactive oxygen species accumulation, and impaired normal processes, such as growth, behavior, and lifespan. The viability of lonp-1 mutants was dependent on the activity of the ATFS-1 transcription factor, and loss of LONP-1 evok

SUBMITTER: Taouktsi E 

PROVIDER: S-EPMC9025075 | biostudies-literature | 2022 Apr

REPOSITORIES: biostudies-literature