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EGFR activation triggers cellular hypertrophy and lysosomal disease in NAGLU-depleted cardiomyoblasts, mimicking the hallmarks of mucopolysaccharidosis IIIB
Mucopolysaccharidosis (MPS) IIIB is an inherited lysosomal storage disease caused by the deficiency of the enzyme α-N-acetylglucosaminidase (NAGLU) required for heparan sulfate (HS) degradation. The defective lysosomal clearance of undigested HS results in dysfunction of multiple tissues and organs....
Autores principales: | De Pasquale, Valeria, Pezone, Antonio, Sarogni, Patrizia, Tramontano, Alfonso, Schiattarella, Gabriele Giacomo, Avvedimento, Vittorio Enrico, Paladino, Simona, Pavone, Luigi Michele |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Nature Publishing Group UK
2018
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5833457/ https://www.ncbi.nlm.nih.gov/pubmed/29348482 http://dx.doi.org/10.1038/s41419-017-0187-0 |
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