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

The Gait Variability Index: A new way to quantify fluctuation magnitude of spatiotemporal parameters during gait

The Gait Variability Index: A new way to quantify fluctuation magnitude of spatiotemporal parameters during gait. Arnaud Gouelle, Fabrice Mégrot, Ana Presedo, Isabelle Husson, Alain Yelnik, Georges-François Penneçot. Gait & Posture, Available online 8 February 2013. http://dx.doi.org/10.1016/j.gaitpost.2013.01.013

Keywords: Gait variability, Fluctuation magnitude, Spatiotemporal parameters, Friedreich's Ataxia

Hypoxia inducible factor prolyl hydroxylases as targets for neuroprotection by “antioxidant” metal chelators: From ferroptosis to stroke

Hypoxia inducible factor prolyl hydroxylases as targets for neuroprotection by “antioxidant” metal chelators: From ferroptosis to stroke. Rachel E. Speer, Saravanan S. Karuppagounder, Manuela Basso, Sama Sleiman, Amit Kumar, David Brand, Natalya Smirnova, Irina Gazaryan, Soah J. Khim, Rajiv R. Ratan. Free Radical Biology and Medicine, Available online 31 January 2013. http://dx.doi.org/10.1016/j.freeradbiomed.2013.01.026

Keywords: Metal chelators, Neurodegeneration, Hypoxia inducible factors, Transcription, Prolyl hydroxylases.

See also:
Frataxin participates to the hypoxia-induced response in tumors
Neuroprotective Multifunctional Iron Chelators: From Redox-Sensitive Process to Novel Therapeutic Opportunities

Mesenchymal stem cells exert a remarkable regenerative effect requiring minimal CNS integration

Mesenchymal stem cells exert a remarkable regenerative effect requiring minimal CNS integration. Antonio Uccelli, Experimental Neurology, Available online 4 February 2013.

The concept of repair of the nervous system by adult stem cells has recently evolved from the original hypothesis that tissue regeneration could have been achieved through stem cells differentiation into neural cells to the current vision that they act mainly by means of paracrine mechanisms. Is preferable the stem cells transplantation or the administration of their secreted factors?.

Keywords: Mesenchymal stem cells, microglia, chemokines, neuroprotection, immunomodulation

The epidemiology of intermittent and chronic ataxia in children in Manitoba, Canada

The epidemiology of intermittent and chronic ataxia in children in Manitoba, Canada. Michael S Salman, Esther J Lee, Anindita Tjahjadi and Bernard N Chodirker. Developmental Medicine & Child Neurology, Article first published online: 7 FEB 2013 | DOI: 10.1111/dmcn.12081

Keywords: retrospective study, age 0–16y, from 1991 to 2008, 184 patients, Angelman syndrome (n=16), ataxia telangiectasia (n=13), mitochondrial disease (n=9), Friedreich ataxia (n=7), stroke (n=7), familial/genetic episodic ataxia (n=7).