Mitochondrial proteases and protein quality control in ageing and longevity

Mitochondrial proteases and protein quality control in ageing and longevity. Marie-Paule Hamon, Anne-Laure Bulteau, Bertrand Friguet, Ageing Research Reviews, Available online 8 January 2015, ISSN 1568-1637, http://dx.doi.org/10.1016/j.arr.2014.12.010.

Concomitant increased Lon and ClpP levels and loss of mitochondrial Fe-S proteins have been found in the muscle creatine kinase mouse heart model for Friedreich ataxia. To our knowledge, no Lon mutation has been identified to date in patients enabling the determination of a phenotype associated with a Lon defect. Nevertheless, its involvement in age-related and other diseases may consider using it as target for anti-ageing strategy or disease treatments.

Variations in ClpP levels have been noted in hereditary spastic paraplegia and Friedreich ataxia. As for Lon, due to its its involvement in age-related and other diseases, the Clp protease may be considered as a target for disease treatments or anti-ageing strategy.