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Mucopolysaccharidosis (MPS IIIA) mice have increased lung compliance and airway resistance, decreased diaphragm strength, and no change in alveolar structure.
Paget TL, Larcombe AN, Pinniger GJ, Tsioutsias I, Schneider JP, Parkinson-Lawrence EJ, Orgeig S. Paget TL, et al. Among authors: tsioutsias i. Am J Physiol Lung Cell Mol Physiol. 2024 Jun 1;326(6):L713-L726. doi: 10.1152/ajplung.00445.2022. Epub 2024 Mar 5. Am J Physiol Lung Cell Mol Physiol. 2024. PMID: 38469649