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The Murine Model of Mucopolysaccharidosis IIIB Develops Cardiopathies over Time Leading to Heart Failure
Mucopolysaccharidosis (MPS) IIIB is a lysosomal disease due to the deficiency of the enzyme α-N-acetylglucosaminidase (NAGLU) required for heparan sulfate (HS) degradation. The disease is characterized by mild somatic features and severe neurological disorders. Very little is known on the cardiac dy...
Autores principales: | Schiattarella, Gabriele Giacomo, Cerulo, Giuliana, De Pasquale, Valeria, Cocchiaro, Pasquale, Paciello, Orlando, Avallone, Luigi, Belfiore, Maria Paola, Iacobellis, Francesca, Di Napoli, Daniele, Magliulo, Fabio, Perrino, Cinzia, Trimarco, Bruno, Esposito, Giovanni, Di Natale, Paola, Pavone, Luigi Michele |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Public Library of Science
2015
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4493027/ https://www.ncbi.nlm.nih.gov/pubmed/26147524 http://dx.doi.org/10.1371/journal.pone.0131662 |
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