Antioxidant effect of cysteamine in brain cortex of young rats

Cysteamine is a cystine-depleting drug used in the treatment of cystinosis, a metabolic disorder caused by deficiency of the lysosomal cystine carrier. As a result, cystine accumulates within lysosomes in many tissues and organs, including the nervous system. Studies with cystine dimethyl ester load...

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Detalhes bibliográficos
Autores: Kessler, Adriana, Wyse, Angela Terezinha de Souza, Biasibetti, Micheli, Melo, Denizar Alberto da Silva, Wajner, Moacir, Dutra Filho, Carlos Severo, Wannmacher, Clovis Milton Duval
Formato: artículo
Estado:Versión publicada
Fecha de publicación:2008
País:Brasil
Recursos:Universidade Federal do Rio Grande do Sul (UFRGS)
Repositorio:Repositório Institucional da UFRGS
Idioma:inglés
OAI Identifier:oai:www.lume.ufrgs.br:10183/218294
Acesso em linha:http://hdl.handle.net/10183/218294
Access Level:acceso abierto
Palavra-chave:Cistinose
Estresse oxidativo
Córtex cerebral
Cysteamine
Cystinosis
Cystine dimethyl ester
Oxidative stress
Brain cortex
Descrição
Resumo:Cysteamine is a cystine-depleting drug used in the treatment of cystinosis, a metabolic disorder caused by deficiency of the lysosomal cystine carrier. As a result, cystine accumulates within lysosomes in many tissues and organs, including the nervous system. Studies with cystine dimethyl ester loaded cells suggest that cystine might induce apoptosis through oxidative stress. Our objective was to investigate the effects of co-administration of cysteamine with the oxidant cystine dimethyl ester on several parameters of oxidative stress in the brain cortex of rats. Animals were injected with 1.6 lmol/g cystine dimethyl ester and/or 0.26 lmol/g body weight cysteamine. Cystine dimethyl ester induced lipoperoxidation, protein carbonylation, and stimulated superoxide dismutase, glutathione peroxidase and catalase activities, probably through the formation of free radicals. Cysteamine prevented those effects, possibly increasing cellular thiol pool and acting as a scavenger of free radicals. These results suggest that the antioxidant effect of cysteamine may be important in the treatment of cystinosis.