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Hepatic but not brain iron is rapidly chelated by deferasirox in aceruloplasminemia due to a novel gene mutation
BACKGROUND & AIMS: Aceruloplasminemia is a rare autosomal recessive neurodegenerative disease associated with brain and liver iron accumulation which typically presents with movement disorders, retinal degeneration, and diabetes mellitus. Ceruloplasmin is a multi-copper ferroxidase that is secre...
Autores principales: | Finkenstedt, Armin, Wolf, Elisabeth, Höfner, Elmar, Gasser, Bethina Isasi, Bösch, Sylvia, Bakry, Rania, Creus, Marc, Kremser, Christian, Schocke, Michael, Theurl, Milan, Moser, Patrizia, Schranz, Melanie, Bonn, Guenther, Poewe, Werner, Vogel, Wolfgang, Janecke, Andreas R., Zoller, Heinz |
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Formato: | Texto |
Lenguaje: | English |
Publicado: |
Elsevier
2010
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2987498/ https://www.ncbi.nlm.nih.gov/pubmed/20801540 http://dx.doi.org/10.1016/j.jhep.2010.04.039 |
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