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Mucopolysaccharidosis (MPS IIIA) mice have increased lung compliance and airways resistance, decreased diaphragm strength and no change in alveolar structure.
Am J Physiol Lung Cell Mol Physiol. 2024 Mar 5. doi: 10.1152/ajplung.00445.2022. Online ahead of print.
Am J Physiol Lung Cell Mol Physiol. 2024.
PMID: 38469649
Increased Alveolar Heparan Sulphate and Reduced Pulmonary Surfactant Amount and Function in the Mucopolysaccharidosis IIIA Mouse.
Paget TL, Parkinson-Lawrence EJ, Trim PJ, Autilio C, Panchal MH, Koster G, Echaide M, Snel MF, Postle AD, Morrison JL, Pérez-Gil J, Orgeig S.
Paget TL, et al.
Cells. 2021 Apr 8;10(4):849. doi: 10.3390/cells10040849.
Cells. 2021.
PMID: 33918094
Free PMC article.
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