Upregulation of the Mitochondrial Lon Protease Allows Adaptation to Acute Oxidative Stress but Dysregulation is Associated with Chronic Stress, Disease, and Aging

Upregulation of the Mitochondrial Lon Protease Allows Adaptation to Acute Oxidative Stress but Dysregulation is Associated with Chronic Stress, Disease, and Aging. Jenny K. Ngo, Laura C.D. Pomatto, Kelvin J.A. Davies. Redox Biology, Available online 9 February 2013. http://dx.doi.org/10.1016/j.redox.2013.01.015.

Keywords: Adaptation, Hormesis, Lon Protease, Protein Degradation and Oxidation, Mitochondria, Oxidative Stress.


In a Friedreich Ataxia mouse model, in which frataxin has been deleted in striated muscles, an increase in ClpP and Lon mRNA, protein, and activity was observed in the isolated mitochondria of mice between 5 and 10 weeks of age [55]. The upregulation of Lon and ClpP was accompanied by a progressive loss of mitochondrial Fe-S proteins with no change in mRNA levels, suggesting degradation.

See also: Frataxin deficiency causes upregulation of mitochondrial Lon and ClpP proteases and severe loss of mitochondrial Fe-S proteins