Catalase localization in Duchenne-Becker patients’ erythrocytes

Abstract

Duchenne-Becker muscular dystrophy (DBMD) might be caused by a widespread genetic defect in surface membranes, which could be expressed in membranes not pathologically involved in DBMD. This hypothesis was supported by a substantial amount of evidence of abnormalities in erythrocytes from patients with DBMD. Catalases are well studied enzymes that play critical roles in protecting cells against the toxic effects of hydrogen peroxide. In previous years a lot of papers on oxidative status in DBMD are mostly confirmed increased activity of enzymes involved in the elimination of reactive oxygen species in order to protect the cells from damage, including superoxide dismutase, catalase and glutathione peroxidase. Immunohistochemistry and immunogold labeling were used to study erythrocytes from patients with Duchenne-Becker muscular dystrophy and from age-matched normal boys. There were significant differences in the catalase localization of erythrocytes from Duchenne patients when compared to controls. Hence, the internal catalase localization in the erythrocyte is atypical in DBMD, supporting the concept that a membrane and cytoskeletal defect involving multiple tissues is present in this disorder.