Studies conducted at University of Porto on cystinosis recently published
2007 SEP 18 -- "Cystinosis is a systemic genetic disease caused by a lysosomal transport deficiency accumulating cystine in most tissues. Although tissue damage might depend on cystine accumulation, the mechanisms of tissue damage are not fully understood," investigators in Porto Alegre, Brazil report. "Studies performed in fibroblasts of cystinotic patients and in kidney cells loaded with cystine dimethyl ester (CDME) suggest that apoptosis is enhanced in this disease. Considering that oxidative stress is a known apoptosis inducer, our main objective was to investigate the effects of CDME loading on several parameters of oxidative stress in the kidney of young rats. Animals were injected twice a day with 1.6 mu mol/g body weight CDME and/or 0.26 mu mol/g body weight cysteamine (CSH) from the 16th to the 20th postpartum day and killed after 1 or 12 h. CDME induced lipoperoxidation and protein carbonylation and stimulated superoxide dismutase, glutathione peroxidase (GPx), and catalase activities, probably through the formation of superoxide anions, hydrogen peroxide, and hydroxyl free radicals. Coadministration of CSH, the drug used to treat cystinotic patients, prevented, at least in part, those effects, possibly acting as a scavenger of free radicals," wrote V.C. Rech and colleagues, University of Porto. The researchers concluded: "These results suggest that the induction of oxidative stress might be one of the mechanisms leading to tissue damage in cystinotic patients." Rech and colleagues published their study in Pediatric Nephrology (Promotion of oxidative stress in kidney of rats loaded with cystine dimethyl ester. Pediatric Nephrology, 2007;22(8):1121-1128). For additional information, contact C.M.D. Wannmacher, University of Porto Alegre, Institute Ciencias Basicas Saude, Dept. of Bioquim, Rua Ramiro Barcelos 2600, BR-90035003 Porto Alegre, RS, Brazil. The publisher of the journal Pediatric Nephrology can be contacted at: Springer, 233 Spring Street, New York, NY 10013, USA. Keywords: Brazil, Porto Alegre, Apoptosis, Cystinosis, Nephrology, Pediatric, University of Porto. This article was prepared by Life Science Weekly editors from staff and other reports. Copyright 2007, Life Science Weekly via NewsRx.com.
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