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Oxidative stress and the altered reaction to it in Fabry disease: A possible target for cardiovascular-renal remodeling?
BACKGROUND: Fabry disease is characterized by deficient expression/activity of α-GalA with consequent lysosomal accumulation in various organs of its substrate Gb3. Despite enzyme replacement therapy, Fabry disease progresses with serious myocardial, cerebral and renal manifestations. Gb3 accumulati...
Autores principales: | Ravarotto, Verdiana, Carraro, Gianni, Pagnin, Elisa, Bertoldi, Giovanni, Simioni, Francesca, Maiolino, Giuseppe, Martinato, Matteo, Landini, Linda, Davis, Paul A., Calò, Lorenzo A. |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Public Library of Science
2018
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6160124/ https://www.ncbi.nlm.nih.gov/pubmed/30261035 http://dx.doi.org/10.1371/journal.pone.0204618 |
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