Biomolecules damage and redox status abnormalities in Fabry patients before and during enzyme replacement therapy
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- 作者：Giovana Brondani Biancinia ; b ; giovana.bb@gmail.com" class="auth_mail" title="E-mail the corresponding author ; Carlos Eduardo Jacquesb ; c ; Tatiane Hammerschmidtb ; Heryk Motta de Souzab ; Bruna Donidaa ; b ; Marion Deonb ; Filippo Pinto Vairob ; Charles Marques Lourenç ; od ; Roberto Giugliania ; b ; e ; Carmen Regla Vargasa ; b ; c ; crvargas@hcpa.edu.br" class="auth_mail" title="E-mail the corresponding author
- 关键词：α-gal A ; α-galactosidase A ; Cr ; creatinine ; ERT ; enzyme replacement therapy ; FD ; Fabry disease ; GCL ; glutamate cysteine ligase ; Gb3 ; globotriaosylceramide ; GLA ; α-galactosidase A coding gene ; GPx ; glutathione peroxidase ; GR ; glutathione reductase ; GSH ; reduced glutathione ; GSSG ; oxidized glutathione ; H2O2 ; hydrogen peroxide ; Lyso-Gb3 ; globotriaosylsphingosine ; MDA ; malondialdehyde ; NO3&minus ; ; nitrate ; NO. ; nitric oxide ; NO2&minus ; ; nitrite ; ONOO&minus ; ; peroxinytrite ; RNS ; rea
- 刊名：Clinica Chimica Acta
- 出版年：2016
- 出版时间：1 October 2016
- 年：2016
- 卷：461
- 期：Complete
- 页码：41-46
- 全文大小：546 K
文摘
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Fabry patients at diagnosis and during long-term ERT were studied.
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Patients present altered oxidative and nitrosative stress profile even before ERT.
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Glutathione metabolism is altered as an adaptive response to stress.
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Lipid peroxidation and nitric oxide equivalents remain higher after ERT.
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Urinary Gb3 decrease after ERT but not to control levels.