Substrate accumulation and extracellular matrix remodelling promote persistent upper airway disease in mucopolysaccharidosis patients on enzyme replacement therapy

INTRODUCTION: Mucopolysaccharide diseases are a group of lysosomal storage disorders caused by deficiencies of hydrolase enzymes, leading to pathological glycosaminoglycan accumulation. A number of mucopolysaccharidosis (MPS) types are characterised by severe airway disease, the aetiology of which i...

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Bibliographic Details
Published in:PLoS One
Main Authors: Pal, Abhijit Ricky, Mercer, Jean, Jones, Simon A., Bruce, Iain A., Bigger, Brian W.
Format: Artigo
Language:Inglês
Published: Public Library of Science 2018
Subjects:
Research Article
Online Access:https://ncbi.nlm.nih.gov/pmc/articles/PMC6143186/
https://ncbi.nlm.nih.gov/pubmed/30226843
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1371/journal.pone.0203216
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