Proteomic analysis demonstrates the role of the quality control protease LONP1 in mitochondrial protein aggregation.

ABSTRACT: The mitochondrial matrix protease LONP1 is an essential part of the organellar protein quality control system. LONP1 has been shown to be involved in respiration control and apoptosis. Furthermore, a reduction in LONP1 level correlates with aging. Up to now, the effects of a LONP1 defect were mostly studied by utilizing transient, siRNA-mediated knockdown approaches. We generated a new cellular model system for studying the impact of LONP1 on mitochondrial protein homeostasis by a CRISPR/Cas-mediated genetic knockdown (gKD). These cells showed a stable reduction of LONP1 along with a mild phenotype characterized by absent morphological differences and only small negative effects on mitochondrial functions under normal culture conditions. To assess the consequences of a permanent LONP1 depl

SUBMITTER: Pollecker K 

PROVIDER: S-EPMC8503632 | biostudies-literature | 2021 Oct

REPOSITORIES: biostudies-literature