Cell stress management by the mitochondrial LonP1 protease – Insights into mitigating developmental, oncogenic and cardiac stress

Sundararajan Venkatesh, Carolyn K. Suzuki

Research output: Contribution to journalReview articlepeer-review

Abstract

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, 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.

Original languageEnglish (US)
Pages (from-to)46-61
Number of pages16
JournalMitochondrion
Volume51
DOIs
StatePublished - Mar 2020

All Science Journal Classification (ASJC) codes

  • Molecular Medicine
  • Molecular Biology
  • Cell Biology

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