Friedreich's footprint. Steven Goodrick, The Lancet Neurology, Volume 14, Issue 2, February 2015, Page 141, ISSN 1474-4422, http://dx.doi.org/10.1016/S1474-4422(15)70005-0.
We can only hope that the time to a treatment or cure will not be so long.
Monday, January 12, 2015
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.
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.
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