{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,18]],"date-time":"2026-07-18T23:05:46Z","timestamp":1784415946958,"version":"3.55.0"},"reference-count":119,"publisher":"MDPI AG","issue":"4","license":[{"start":{"date-parts":[[2021,4,8]],"date-time":"2021-04-08T00:00:00Z","timestamp":1617840000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cells"],"abstract":"<jats:p>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 investigated in MPS IIIA. We measured heparan sulphate (HS), lipids and surfactant proteins (SP) in pulmonary tissue and bronchoalveolar lavage fluid (BALF), and surfactant activity in healthy and diseased mice (20 weeks of age). Heparan sulphate, ganglioside GM3 and bis(monoacylglycero)phosphate (BMP) were increased in MPS IIIA lung tissue. There was an increase in HS and a decrease in BMP and cholesteryl esters (CE) in MPS IIIA BALF. Phospholipid composition remained unchanged, but BALF total phospholipids were reduced (49.70%) in MPS IIIA. There was a reduction in SP-A, -C and -D mRNA, SP-D protein in tissue and SP-A, -C and -D protein in BALF of MPS IIIA mice. Captive bubble surfactometry showed an increase in minimum and maximum surface tension and percent surface area compression, as well as a higher compressibility and hysteresis in MPS IIIA surfactant upon dynamic cycling. Collectively these biochemical and biophysical changes in alveolar surfactant are likely to be detrimental to lung function in MPS IIIA.<\/jats:p>","DOI":"10.3390\/cells10040849","type":"journal-article","created":{"date-parts":[[2021,4,8]],"date-time":"2021-04-08T13:23:42Z","timestamp":1617888222000},"page":"849","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Increased Alveolar Heparan Sulphate and Reduced Pulmonary Surfactant Amount and Function in the Mucopolysaccharidosis IIIA Mouse"],"prefix":"10.3390","volume":"10","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3664-8752","authenticated-orcid":false,"given":"Tamara L.","family":"Paget","sequence":"first","affiliation":[{"name":"Mechanisms in Cell Biology and Disease Group, UniSA Clinical and Health Sciences, University of South Australia, Adelaide, SA 5000, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emma J.","family":"Parkinson-Lawrence","sequence":"additional","affiliation":[{"name":"Mechanisms in Cell Biology and Disease Group, UniSA Clinical and Health Sciences, University of South Australia, Adelaide, SA 5000, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul J.","family":"Trim","sequence":"additional","affiliation":[{"name":"Proteomics, Metabolomics and MS-Imaging Core Facility, South Australian Health and Medical Research Institute, Adelaide, SA 5000, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chiara","family":"Autilio","sequence":"additional","affiliation":[{"name":"Department of Biochemistry, Faculty of Biology and Research Institute Hospital 12 de Octubre (Imas12), Complutense University, 28003 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Madhuriben H.","family":"Panchal","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, University of Southampton, Southampton SO16 6YD, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Grielof","family":"Koster","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, University of Southampton, Southampton SO16 6YD, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mercedes","family":"Echaide","sequence":"additional","affiliation":[{"name":"Department of Biochemistry, Faculty of Biology and Research Institute Hospital 12 de Octubre (Imas12), Complutense University, 28003 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marten F.","family":"Snel","sequence":"additional","affiliation":[{"name":"Proteomics, Metabolomics and MS-Imaging Core Facility, South Australian Health and Medical Research Institute, Adelaide, SA 5000, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anthony D.","family":"Postle","sequence":"additional","affiliation":[{"name":"Faculty of Medicine, University of Southampton, Southampton SO16 6YD, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Janna L.","family":"Morrison","sequence":"additional","affiliation":[{"name":"Early Origins Adult Health Research Group, Health and Biomedical Innovation, UniSA Clinical and Health Sciences, University of South Australia, Adelaide, SA 5000, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3587-7147","authenticated-orcid":false,"given":"J\u00e9sus","family":"P\u00e9rez-Gil","sequence":"additional","affiliation":[{"name":"Department of Biochemistry, Faculty of Biology and Research Institute Hospital 12 de Octubre (Imas12), Complutense University, 28003 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sandra","family":"Orgeig","sequence":"additional","affiliation":[{"name":"Mechanisms in Cell Biology and Disease Group, UniSA Clinical and Health Sciences, University of South Australia, Adelaide, SA 5000, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2021,4,8]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"241","DOI":"10.1002\/path.3021","article-title":"The cellular pathology of lysosomal diseases","volume":"226","author":"Cox","year":"2012","journal-title":"J. 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