Promotion of oxidative stress in kidney of rats loaded with cystine dimethyl ester

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• 作者：Virgínia Cielo Rech ; Luciane Rosa Feksa ; Maria Fernanda Arevalo do Amaral ; Gustavo Waltereith Koch ; Moacir Wajner ; Carlos Severo Dutra-Filho ; Angela Terezinha de Souza Wyse and Clovis Milton Duval Wannmacher
• 关键词：Cystinosis ; Cystine dimethyl ester ; Cystine ; Oxidative stress ; Kidney
• 刊名：Pediatric Nephrology
• 出版年：2007
• 出版时间：August, 2007
• 年：2007
• 卷：22
• 期：8
• 页码：1121-1128
• 全文大小：264 KB
• 刊物类别：Medicine
• 刊物主题：Medicine & Public Health
  Pediatrics
  
• 出版者：Springer Berlin / Heidelberg
• ISSN：1432-198X

文摘

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. 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 μmol/g body weight CDME and/or 0.26 μmol/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. These results suggest that the induction of oxidative stress might be one of the mechanisms leading to tissue damage in cystinotic patients.