Apoptosis and ROS detoxification enzymes correlate with cytochrome c oxidase deficiency in mitochondrial encephalomyopathies
S Di Giovanni, M Mirabella, M Papacci… - Molecular and Cellular …, 2001 - Elsevier
The aim of this work was to investigate in muscle the role of apoptosis and of oxidative stress
in mitochondrial disorders with dysfunction of respiratory chain. In patients with cytochrome c
oxidase deficiency (COX) we found a variable number of myofibers with apoptotic nuclei that
matched with the level of enzymatic reduction and roughly correlated with muscle weakness.
In parallel, a positive immunostaining for apoptosis-related proteins and Mn and Cu/Zn
superoxide dismutase (SOD) were mostly localized in COX-negative fibers. Moreover …
in mitochondrial disorders with dysfunction of respiratory chain. In patients with cytochrome c
oxidase deficiency (COX) we found a variable number of myofibers with apoptotic nuclei that
matched with the level of enzymatic reduction and roughly correlated with muscle weakness.
In parallel, a positive immunostaining for apoptosis-related proteins and Mn and Cu/Zn
superoxide dismutase (SOD) were mostly localized in COX-negative fibers. Moreover …