Increased Alveolar Heparan Sulphate and Reduced Pulmonary Surfactant Amount and Function in the Mucopolysaccharidosis IIIA Mouse

Increased Alveolar Heparan Sulphate and Reduced Pulmonary Surfactant Amount and Function in the Mucopolysaccharidosis IIIA Mouse

Mucopolysaccharidosis IIIA (MPS IIIA) is a lysosomal storage disease with significant neurological and skeletal pathologies. Respiratory dysfunction is a secondary pathology contributing to mortality in MPS IIIA patients. Pulmonary surfactant is crucial to optimal lung function and has not been inve...

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Bibliographic Details
Main Authors: Tamara L. Paget, Emma J. Parkinson-Lawrence, Paul J. Trim, Chiara Autilio, Madhuriben H. Panchal, Grielof Koster, Mercedes Echaide, Marten F. Snel, Anthony D. Postle, Janna L. Morrison, Jésus Pérez-Gil, Sandra Orgeig
Format: Article
Language:English
Published: MDPI AG 2021-04-01
Series:Cells
Subjects:
MPS IIIA
Sanfilippo syndrome
pulmonary surfactant
respiratory dysfunction
heparan sulphate
bronchoalveolar lavage
Online Access:https://www.mdpi.com/2073-4409/10/4/849

Internet

https://www.mdpi.com/2073-4409/10/4/849

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