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...

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Main Authors: Gabriele Giacomo Schiattarella, Giuliana Cerulo, Valeria De Pasquale, Pasquale Cocchiaro, Orlando Paciello, Luigi Avallone, Maria Paola Belfiore, Francesca Iacobellis, Daniele Di Napoli, Fabio Magliulo, Cinzia Perrino, Bruno Trimarco, Giovanni Esposito, Paola Di Natale, Luigi Michele Pavone
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2015-01-01
Series:PLoS ONE
Online Access:http://europepmc.org/articles/PMC4493027?pdf=render