Impaired enzymatic defensive activity, mitochondrial dysfunction and proteasome activation are involved in RTT cell oxidative damage.

Abstract

A strong correlation between oxidative stress (OS) and Rett syndrome (RTT), a rare neurodevelopmental disorder affecting females in the 95% of the cases, has been well documented although the source of OS and the effect of a redox imbalance in this pathology has not been yet investigated. Using freshly isolated skin fibroblasts from RTT patients and healthy… (More)
DOI: 10.1016/j.bbadis.2015.07.014

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Cite this paper

@article{Cervellati2015ImpairedED, title={Impaired enzymatic defensive activity, mitochondrial dysfunction and proteasome activation are involved in RTT cell oxidative damage.}, author={Carlo Cervellati and Claudia Sticozzi and Arianna Romani and Giuseppe Belmonte and Domenico de Rasmo and Anna Lisa Signorile and Franco Cervellati and Chiara Milanese and Pier G. Mastroberardino and Alessandra Pecorelli and Vinno Savelli and Henry Jay Forman and Joussef Hayek and Giuseppe Valacchi}, journal={Biochimica et biophysica acta}, year={2015}, volume={1852 10 Pt A}, pages={2066-74} }