Mitochondrial Lon protease in human disease and aging: Including an etiologic classification of Lon-related diseases and disorders

We have witnessed a significant increase in knowledge regarding Lon's involvement in physiological functions, as well as in an expanding array of human disorders, including cancer, neurodegeneration, heart disease, and stroke

Daniela A. Bota; Kelvin J.A. Davies

2016

Scholarcy highlights

  • Mitochondria are essential components of cellular metabolism, and rapid changes in the mitochondrial protein pool are required for adaptation to a variety of conditions, from increased demand for oxidative energy production to hypoxic adaptation
  • Mitochondrial homeostasis is crucial for cell survival, and mitochondrial dysfunction leads to apoptotic cell death in normal cells and to aberrant adaptation and selection of hypoxic phenotypes in pathologic conditions such as cancer
  • Though multiple proteolytic systems are described in mammalian mitochondria, the Lon protease seems to be the dominant enzyme involved in maintaining appropriate mitochondrial function, protecting the mtDNA against a variety of insults and controlling mitochondrial protein stoichiometry
  • New therapies focused on Lon inhibition as a potential target for cancer therapies are currently underway
  • Their development has to be approached with careful attention to possible severe central nervous system and cardiac toxicities – that are potentially associated with chronic Lon down-regulation
  • We have witnessed a significant increase in knowledge regarding Lon's involvement in physiological functions, as well as in an expanding array of human disorders, including cancer, neurodegeneration, heart disease, and stroke
  • The interaction of Lon regulation in aging, with the effects of many age-related pathologies will be a significant challenge for researchers in the field to tackle

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