Early Neurodegeneration Progresses Independently of Microglial Activation by Heparan Sulfate in the Brain of Mucopolysaccharidosis IIIB Mice

BACKGROUND: In mucopolysaccharidosis type IIIB, a lysosomal storage disease causing early onset mental retardation in children, the production of abnormal oligosaccharidic fragments of heparan sulfate is associated with severe neuropathology and chronic brain inflammation. We addressed causative lin...

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Hauptverfasser: Ausseil, Jérôme, Desmaris, Nathalie, Bigou, Stéphanie, Attali, Ruben, Corbineau, Sébastien, Vitry, Sandrine, Parent, Mathieu, Cheillan, David, Fuller, Maria, Maire, Irène, Vanier, Marie-Thérèse, Heard, Jean-Michel
Format: Artigo
Sprache:Inglês
Veröffentlicht: Public Library of Science 2008
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Research Article
Online Zugang:https://ncbi.nlm.nih.gov/pmc/articles/PMC2396504/
https://ncbi.nlm.nih.gov/pubmed/18509511
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1371/journal.pone.0002296
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