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Oxidative Stress in Cystinosis Patients
BACKGROUND/AIMS: Nephropathic cystinosis (NC) is a severe systemic disease and cysteamine improves its prognosis. Lysosomal cystine accumulation is the hallmark of cystinosis and is regarded as the primary defect due to mutations in the CTNS gene. However, there is great evidence that cystine accumu...
Autores principales: | Vaisbich, Maria Helena, Pache de Faria Guimaraes, Luciana, Shimizu, Maria Heloisa Mazzola, Seguro, Antonio Carlos |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
S. Karger AG
2011
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3290844/ https://www.ncbi.nlm.nih.gov/pubmed/22470381 http://dx.doi.org/10.1159/000331445 |
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