Increased Alveolar Heparan Sulphate and Reduced Pulmonary Surfactant Amount and Function in the Mucopolysaccharidosis IIIA Mouse
cells Article Increased Alveolar Heparan Sulphate and Reduced Pulmonary Surfactant Amount and Function in the Mucopolysaccharidosis IIIA Mouse Tamara L. Paget 1 , Emma J. Parkinson-Lawrence 1, Paul J. Trim 2, Chiara Autilio 3, Madhuriben H. Panchal 4, Grielof Koster 4, Mercedes Echaide 3, Marten F. Snel 2, Anthony D. Postle 4, Janna L. Morrison 5,Jésus Pérez-Gil 3 and Sandra Orgeig 1,* 1 Mechanisms in Cell Biology and Disease Group, UniSA Clinical and Health Sciences, University of South Australia, Adelaide, SA 5000, Australia; tamara.paget@mymail.unisa.edu.au (T.L.P.); emma.parkinson-lawrence@unisa.edu.au (E.J.P.-L.) 2 Proteomics, Metabolomics and MS-Imaging Core Facility, South Australian Health and Medical Research Institute, Adelaide, SA 5000, Australia; Paul.Trim@sahmri.com (P.J.T.); Marten.Snel@sahmri.com (M.F.S.) 3 Department of Biochemistry, Faculty of Biology and Research Institute Hospital 12 de Octubre (Imas12), Complutense University, 28003 Madrid, Spain; cautilio@ucm.es (C.A.); mechaide@pdi.ucm.es (M.E.); jperezgil@bio.ucm.es (J.P.-G.) 4 Faculty of Medicine, University of Southampton, Southampton SO16 6YD, UK; M.H.Panchal@soton.ac.uk (M.H.P.); G.Koster@soton.ac.uk (G.K.); A.D.Postle@soton.ac.uk (A.D.P.) 5 Early Origins Adult Health Research Group, Health and Biomedical Innovation, UniSA Clinical and Health Sciences, University of South Australia, Adelaide, SA 5000, Australia; janna.morrison@unisa.edu.au * Correspondence: Sandra.Orgeig@unisa.edu.au; Tel.: +61-410-422-712 Citation: Paget, T.L.; Parkinson-Lawrence, E.J.; Trim, P.J.; Abstract: Mucopolysaccharidosis IIIA (MPS IIIA) is a lysosomal storage disease with significant Autilio, C.; Panchal, M.H.; Koster, G.; Echaide, M.; Snel, M.F.; Postle, A.D.; neurological and skeletal pathologies.
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