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

Abhijit Ricky Pal; Jean Mercer; Simon A. Jones; Iain A. Bruce; Brian W. Bigger

doi:10.1371/journal.pone.0203216

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Open Access

Peer-reviewed

Research Article

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

Abhijit Ricky Pal,

Roles Conceptualization, Data curation, Formal analysis, Investigation, Methodology, Writing – original draft

Affiliations Department of Paediatric Otolaryngology, Royal Manchester Children’s Hospital, Manchester, United Kingdom, Stem Cell and Neurotherapies, Division of Cell Matrix Biology & Regenerative Medicine, Faculty of Biology, Medicine and Health, Manchester Academic Health Science Centre, University of Manchester, Manchester, United Kingdom
⨯
Jean Mercer,

Roles Data curation, Project administration, Resources

Affiliation Willink Biochemical Genetics Unit, Manchester Centre for Genomic Medicine, St. Mary’s Hospital, Manchester, United Kingdom
⨯
Simon A. Jones,

Roles Conceptualization, Funding acquisition, Resources, Supervision

Affiliation Willink Biochemical Genetics Unit, Manchester Centre for Genomic Medicine, St. Mary’s Hospital, Manchester, United Kingdom
⨯
Iain A. Bruce,

Roles Conceptualization, Funding acquisition, Investigation, Resources, Supervision, Writing – review & editing

Affiliations Department of Paediatric Otolaryngology, Royal Manchester Children’s Hospital, Manchester, United Kingdom, Respiratory and Allergy Centre, Institute of Inflammation and Repair, Faculty of Medical and Human Sciences, University of Manchester, Manchester, United Kingdom

http://orcid.org/0000-0003-0831-4760

⨯
Brian W. Bigger

Roles Conceptualization, Funding acquisition, Supervision, Writing – review & editing

* E-mail: brian.bigger@manchester.ac.uk

Affiliation Stem Cell and Neurotherapies, Division of Cell Matrix Biology & Regenerative Medicine, Faculty of Biology, Medicine and Health, Manchester Academic Health Science Centre, University of Manchester, Manchester, United Kingdom

http://orcid.org/0000-0002-9708-1112

⨯

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

Abhijit Ricky Pal,
Jean Mercer,
Simon A. Jones,
Iain A. Bruce,
Brian W. Bigger

Published: September 18, 2018
https://doi.org/10.1371/journal.pone.0203216

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