{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,9]],"date-time":"2026-07-09T06:13:44Z","timestamp":1783577624135,"version":"3.55.0"},"reference-count":49,"publisher":"Elsevier BV","issue":"1-2","license":[{"start":{"date-parts":[[2025,9,1]],"date-time":"2025-09-01T00:00:00Z","timestamp":1756684800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2025,9,1]],"date-time":"2025-09-01T00:00:00Z","timestamp":1756684800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2025,9,12]],"date-time":"2025-09-12T00:00:00Z","timestamp":1757635200000},"content-version":"vor","delay-in-days":11,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100020480","name":"CSL Limited","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100020480","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100008227","name":"University of South Africa","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100008227","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100017570","name":"Commonwealth Serum Laboratories","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100017570","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100023560","name":"Bioplatforms Australia","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100023560","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001787","name":"University of South Australia","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100001787","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100015015","name":"Government of South Australia","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100015015","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Molecular Genetics and Metabolism"],"published-print":{"date-parts":[[2025,9]]},"DOI":"10.1016\/j.ymgme.2025.109231","type":"journal-article","created":{"date-parts":[[2025,9,7]],"date-time":"2025-09-07T14:26:18Z","timestamp":1757255178000},"page":"109231","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":2,"title":["Defining lung pathogenesis in a murine model of mucopolysaccharidosis Type I by proteomic analysis"],"prefix":"10.1016","volume":"146","author":[{"given":"Yuen T.","family":"Ngai","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Clifford","family":"Young","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emma J.","family":"Parkinson-Lawrence","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sabine","family":"Wimmer-Kleikamp","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Parul","family":"Mittal","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Helen","family":"Beard","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthew T.","family":"Briggs","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Manuela","family":"Klingler-Hoffmann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Doug A.","family":"Brooks","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sandra","family":"Orgeig","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"Hoffmann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"issue":"s455","key":"10.1016\/j.ymgme.2025.109231_bb0005","doi-asserted-by":"crossref","first-page":"26","DOI":"10.1111\/j.1651-2227.2007.00202.x","article-title":"Pathogenic mechanisms in lysosomal disease: a reappraisal of the role of the lysosome","volume":"96","author":"Walkley","year":"2007","journal-title":"Acta Paediatr."},{"issue":"1","key":"10.1016\/j.ymgme.2025.109231_bb0010","doi-asserted-by":"crossref","first-page":"27","DOI":"10.1038\/s41572-018-0025-4","article-title":"Lysosomal storage diseases","volume":"4","author":"Platt","year":"2018","journal-title":"Nat. Rev. Dis. Primers"},{"key":"10.1016\/j.ymgme.2025.109231_bb0015","doi-asserted-by":"crossref","first-page":"24","DOI":"10.1186\/1750-1172-3-24","article-title":"The prevalence of and survival in mucopolysaccharidosis I: hurler, hurler-Scheie and Scheie syndromes in the UK","volume":"3","author":"Moore","year":"2008","journal-title":"Orphanet J. Rare Dis."},{"key":"10.1016\/j.ymgme.2025.109231_bb0020","series-title":"GeneReviews(\u00ae)","article-title":"Mucopolysaccharidosis type I","author":"Clarke","year":"1993"},{"issue":"2","key":"10.1016\/j.ymgme.2025.109231_bb0025","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1016\/j.prrv.2010.10.005","article-title":"Respiratory manifestations in mucopolysaccharidoses","volume":"12","author":"Muhlebach","year":"2011","journal-title":"Paediatr. Respir. Rev."},{"issue":"1","key":"10.1016\/j.ymgme.2025.109231_bb0030","doi-asserted-by":"crossref","first-page":"74","DOI":"10.1038\/s41434-019-0113-4","article-title":"Neonatal nonviral gene editing with the CRISPR\/Cas9 system improves some cardiovascular, respiratory, and bone disease features of the mucopolysaccharidosis I phenotype in mice","volume":"27","author":"Schuh","year":"2020","journal-title":"Gene Ther."},{"issue":"1","key":"10.1016\/j.ymgme.2025.109231_bb0035","doi-asserted-by":"crossref","first-page":"32","DOI":"10.1186\/s13023-017-0583-7","article-title":"Early disease progression of hurler syndrome","volume":"12","author":"Kiely","year":"2017","journal-title":"Orphanet J. Rare Dis."},{"issue":"10","key":"10.1016\/j.ymgme.2025.109231_bb0040","doi-asserted-by":"crossref","first-page":"759","DOI":"10.1038\/gim.2014.25","article-title":"The natural history of MPS I: global perspectives from the MPS I registry","volume":"16","author":"Beck","year":"2014","journal-title":"Genet. Med."},{"issue":"2","key":"10.1016\/j.ymgme.2025.109231_bb0045","doi-asserted-by":"crossref","first-page":"146","DOI":"10.1016\/j.ymgme.2014.09.012","article-title":"Extracellular matrix disruption is an early event in the pathogenesis of skeletal disease in mucopolysaccharidosis I","volume":"114","author":"Heppner","year":"2015","journal-title":"Mol. Genet. Metab."},{"issue":"4","key":"10.1016\/j.ymgme.2025.109231_bb0050","doi-asserted-by":"crossref","first-page":"281","DOI":"10.1111\/cge.13583","article-title":"Genotype-phenotype relationships in mucopolysaccharidosis type I (MPS I): insights from the international MPS I registry","volume":"96","author":"Clarke","year":"2019","journal-title":"Clin. Genet."},{"issue":"15","key":"10.1016\/j.ymgme.2025.109231_bb0055","doi-asserted-by":"crossref","first-page":"7888","DOI":"10.3390\/ijms22157888","article-title":"Differences in MPS I and MPS II disease manifestations","volume":"22","author":"Hampe","year":"2021","journal-title":"Int. J. Mol. Sci."},{"issue":"6","key":"10.1016\/j.ymgme.2025.109231_bb0060","doi-asserted-by":"crossref","first-page":"911","DOI":"10.1007\/s00431-011-1644-x","article-title":"Diagnosis and treatment trends in mucopolysaccharidosis I: findings from the MPS I registry","volume":"171","author":"D\u2019Aco","year":"2012","journal-title":"Eur. J. Pediatr."},{"issue":"8","key":"10.1016\/j.ymgme.2025.109231_bb0065","doi-asserted-by":"crossref","DOI":"10.3390\/cells9081838","article-title":"Mucopolysaccharidosis Type I: a review of the natural history and molecular pathology","volume":"9","author":"Hampe","year":"2020","journal-title":"Cells"},{"issue":"2","key":"10.1016\/j.ymgme.2025.109231_bb0070","doi-asserted-by":"crossref","first-page":"189","DOI":"10.3390\/biom11020189","article-title":"Mucopolysaccharidosis Type I: current treatments, limitations, and prospects for improvement","volume":"11","author":"Hampe","year":"2021","journal-title":"Biomolecules"},{"key":"10.1016\/j.ymgme.2025.109231_bb0075","first-page":"63","article-title":"The mucopolysaccharide (MPS) I mouse displays lung and diaphragm dysfunction","volume":"27","author":"Orgeig","year":"2022","journal-title":"Respirology"},{"key":"10.1016\/j.ymgme.2025.109231_bb0080","doi-asserted-by":"crossref","first-page":"1079359","DOI":"10.3389\/fvets.2022.1079359","article-title":"Proteomics approaches: a review regarding an importance of proteome analyses in understanding the pathogens and diseases","volume":"9","author":"Zubair","year":"2022","journal-title":"Front. Vet. Sci."},{"issue":"2","key":"10.1016\/j.ymgme.2025.109231_bb0085","doi-asserted-by":"crossref","DOI":"10.1016\/j.ymgme.2023.107371","article-title":"Proteomics identifies novel biomarkers of synovial joint disease in a canine model of mucopolysaccharidosis I","volume":"138","author":"Zhang","year":"2023","journal-title":"Mol. Genet. Metab."},{"issue":"2","key":"10.1016\/j.ymgme.2025.109231_bb0090","doi-asserted-by":"crossref","first-page":"138","DOI":"10.1016\/j.ymgme.2014.12.301","article-title":"Shotgun proteomics reveals possible mechanisms for cognitive impairment in mucopolysaccharidosis I mice","volume":"114","author":"Baldo","year":"2015","journal-title":"Mol. Genet. Metab."},{"issue":"4","key":"10.1016\/j.ymgme.2025.109231_bb0095","doi-asserted-by":"crossref","DOI":"10.3390\/ijms23042290","article-title":"Lysosomes at the crossroads of cell metabolism, cell cycle, and stemness","volume":"23","author":"Nowosad","year":"2022","journal-title":"Int. J. Mol. Sci."},{"issue":"1","key":"10.1016\/j.ymgme.2025.109231_bb0100","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1186\/s13023-015-0337-3","article-title":"Decreased performance in IDUA knockout mouse mimic limitations of joint function and locomotion in patients with hurler syndrome","volume":"10","author":"Kim","year":"2015","journal-title":"Orphanet J. Rare Dis."},{"issue":"4","key":"10.1016\/j.ymgme.2025.109231_bb0105","doi-asserted-by":"crossref","first-page":"875","DOI":"10.1083\/jcb.104.4.875","article-title":"Rapid analytical and preparative isolation of functional endosomes by free flow electrophoresis","volume":"104","author":"Marsh","year":"1987","journal-title":"J. Cell Biol."},{"issue":"7","key":"10.1016\/j.ymgme.2025.109231_bb0110","doi-asserted-by":"crossref","first-page":"554","DOI":"10.1038\/nrm1423","article-title":"The cell biology of lysosomal storage disorders","volume":"5","author":"Futerman","year":"2004","journal-title":"Nat. Rev. Mol. Cell Biol."},{"issue":"5\u20136","key":"10.1016\/j.ymgme.2025.109231_bb0115","doi-asserted-by":"crossref","first-page":"495","DOI":"10.1016\/j.mam.2006.08.005","article-title":"Roles of LAMP-1 and LAMP-2 in lysosome biogenesis and autophagy","volume":"27","author":"Eskelinen","year":"2006","journal-title":"Mol. Asp. Med."},{"issue":"4","key":"10.1016\/j.ymgme.2025.109231_bb0120","doi-asserted-by":"crossref","first-page":"295","DOI":"10.1016\/j.tibs.2019.12.007","article-title":"Structure and roles of V-type ATPases","volume":"45","author":"Vasanthakumar","year":"2020","journal-title":"Trends Biochem. Sci."},{"issue":"1","key":"10.1016\/j.ymgme.2025.109231_bb0125","doi-asserted-by":"crossref","DOI":"10.3390\/ijms24010477","article-title":"Mucopolysaccharidoses: cellular consequences of glycosaminoglycans accumulation and potential targets","volume":"24","author":"Leal","year":"2022","journal-title":"Int. J. Mol. Sci."},{"issue":"1","key":"10.1016\/j.ymgme.2025.109231_bb0130","doi-asserted-by":"crossref","first-page":"1760","DOI":"10.1038\/s41467-022-29461-8","article-title":"Quantitative phosphoproteomic analyses identify STK11IP as a lysosome-specific substrate of mTORC1 that regulates lysosomal acidification","volume":"13","author":"Zi","year":"2022","journal-title":"Nat. Commun."},{"issue":"6056","key":"10.1016\/j.ymgme.2025.109231_bb0135","doi-asserted-by":"crossref","first-page":"678","DOI":"10.1126\/science.1207056","article-title":"mTORC1 senses lysosomal amino acids through an inside-out mechanism that requires the Vacuolar H<sup>+<\/sup>-ATPase","volume":"334","author":"Zoncu","year":"2011","journal-title":"Science"},{"issue":"1","key":"10.1016\/j.ymgme.2025.109231_bb0140","doi-asserted-by":"crossref","first-page":"28","DOI":"10.1186\/s41232-023-00278-2","article-title":"The Ragulator complex: delving its multifunctional impact on metabolism and beyond","volume":"43","author":"Tsujimoto","year":"2023","journal-title":"Inflamm. Regen."},{"key":"10.1016\/j.ymgme.2025.109231_bb0145","unstructured":"Fan Y, Moser J, Jongman R, et al. Compositional changes of the lung extracellular matrix in acute respiratory distress syndrome. Am. J. Phys. Cell Phys.0(0):null."},{"issue":"4","key":"10.1016\/j.ymgme.2025.109231_bb0150","doi-asserted-by":"crossref","DOI":"10.3390\/cells8040342","article-title":"Extracellular matrix component Remodeling in respiratory diseases: what has been found in clinical and experimental studies?","volume":"8","author":"Ito","year":"2019","journal-title":"Cells"},{"key":"10.1016\/j.ymgme.2025.109231_bb0155","doi-asserted-by":"crossref","DOI":"10.1016\/j.carbpol.2020.117261","article-title":"The abnormal accumulation of heparan sulfate in patients with mucopolysaccharidosis prevents the elastolytic activity of cathepsin V","volume":"253","author":"Chazeirat","year":"2021","journal-title":"Carbohydr. Polym."},{"issue":"19","key":"10.1016\/j.ymgme.2025.109231_bb0160","doi-asserted-by":"crossref","DOI":"10.3390\/ijms231911168","article-title":"MPSI manifestations and treatment outcome: skeletal focus","volume":"23","author":"De Ponti","year":"2022","journal-title":"Int. J. Mol. Sci."},{"issue":"1","key":"10.1016\/j.ymgme.2025.109231_bb0165","doi-asserted-by":"crossref","first-page":"194","DOI":"10.1186\/s12864-024-10049-z","article-title":"Genome-wide identification and structural analysis of the BMP gene family in Triplophysa dalaica","volume":"25","author":"Zhang","year":"2024","journal-title":"BMC Genomics"},{"issue":"4","key":"10.1016\/j.ymgme.2025.109231_bb0170","doi-asserted-by":"crossref","first-page":"858","DOI":"10.1016\/S0006-291X(03)01500-6","article-title":"Heparan sulfate is required for bone morphogenetic protein-7 signaling","volume":"308","author":"Irie","year":"2003","journal-title":"Biochem. Biophys. Res. Commun."},{"issue":"6","key":"10.1016\/j.ymgme.2025.109231_bb0175","first-page":"C1387","article-title":"Endogenous heparan sulfate and heparin modulate bone morphogenetic protein-4 signaling and activity","volume":"294","author":"Khan","year":"2008","journal-title":"Am. J. Phys. Cell Phys."},{"issue":"6","key":"10.1016\/j.ymgme.2025.109231_bb0180","doi-asserted-by":"crossref","first-page":"2954","DOI":"10.1074\/jbc.M115.683292","article-title":"The Prodomain-bound form of bone morphogenetic protein 10 is biologically active on endothelial cells","volume":"291","author":"Jiang","year":"2016","journal-title":"J. Biol. Chem."},{"issue":"9","key":"10.1016\/j.ymgme.2025.109231_bb0185","doi-asserted-by":"crossref","first-page":"912","DOI":"10.2108\/zsj.25.912","article-title":"Molecular cloning and developmental expression of a hyaluronan and proteoglycan link protein gene, <i>crtl1\/hapln1<\/i>, in Zebrafish","volume":"25","author":"Kang","year":"2008","journal-title":"Zool. Sci."},{"issue":"Suppl. 1","key":"10.1016\/j.ymgme.2025.109231_bb0190","doi-asserted-by":"crossref","first-page":"S30","DOI":"10.1513\/AnnalsATS.201406-240MG","article-title":"Lung extracellular matrix and fibroblast function","volume":"12 Suppl 1","author":"White","year":"2015","journal-title":"Ann. Am. Thorac. Soc."},{"issue":"10","key":"10.1016\/j.ymgme.2025.109231_bb0195","doi-asserted-by":"crossref","first-page":"1907","DOI":"10.3390\/cancers16101907","article-title":"Hyaluronic acid interacting molecules mediated crosstalk between Cancer cells and microenvironment from primary tumour to distant metastasis","volume":"16","author":"Xu","year":"2024","journal-title":"Cancers"},{"key":"10.1016\/j.ymgme.2025.109231_bb0200","doi-asserted-by":"crossref","DOI":"10.1016\/j.bbrc.2024.150386","article-title":"Glycoprotein non-metastatic melanoma protein B (GPNMB): an attractive target in atherosclerosis","volume":"732","author":"Yu","year":"2024","journal-title":"Biochem. Biophys. Res. Commun."},{"issue":"1","key":"10.1016\/j.ymgme.2025.109231_bb0205","doi-asserted-by":"crossref","first-page":"136","DOI":"10.1038\/s42003-022-04333-5","article-title":"Macrophage-derived GPNMB trapped by fibrotic extracellular matrix promotes pulmonary fibrosis","volume":"6","author":"Wang","year":"2023","journal-title":"Commun. Biol."},{"issue":"2","key":"10.1016\/j.ymgme.2025.109231_bb0210","doi-asserted-by":"crossref","first-page":"612","DOI":"10.1016\/j.mam.2012.05.011","article-title":"The SLC39 family of zinc transporters","volume":"34","author":"Jeong","year":"2013","journal-title":"Mol. Asp. Med."},{"issue":"1","key":"10.1016\/j.ymgme.2025.109231_bb0215","doi-asserted-by":"crossref","first-page":"18","DOI":"10.1186\/s13041-019-0439-2","article-title":"Lysosomal dysfunction in proteinopathic neurodegenerative disorders: possible therapeutic roles of cAMP and zinc","volume":"12","author":"Koh","year":"2019","journal-title":"Mol. Brain"},{"issue":"1","key":"10.1016\/j.ymgme.2025.109231_bb0220","doi-asserted-by":"crossref","first-page":"27","DOI":"10.2174\/1567201043480036","article-title":"The role of ABC transporters in drug resistance, metabolism and toxicity","volume":"1","author":"Glavinas","year":"2004","journal-title":"Curr. Drug Deliv."},{"issue":"23","key":"10.1016\/j.ymgme.2025.109231_bb0225","doi-asserted-by":"crossref","first-page":"3965","DOI":"10.1002\/1873-3468.13967","article-title":"An inventory of lysosomal ABC transporters","volume":"594","author":"Szakacs","year":"2020","journal-title":"FEBS Lett."},{"key":"10.1016\/j.ymgme.2025.109231_bb0230","doi-asserted-by":"crossref","DOI":"10.1016\/j.drup.2024.101062","article-title":"The role of ABCC10\/MRP7 in anti-cancer drug resistance and beyond","volume":"73","author":"Chen","year":"2024","journal-title":"Drug Resist. Updat."},{"issue":"4","key":"10.1016\/j.ymgme.2025.109231_bb0235","doi-asserted-by":"crossref","first-page":"503","DOI":"10.1093\/hmg\/6.4.503","article-title":"Murine mucopolysaccharidosis Type I: targeted disruption of the murine \u03b1-l-Iduronidase gene","volume":"6","author":"Clarke","year":"1997","journal-title":"Hum. Mol. Genet."},{"issue":"9","key":"10.1016\/j.ymgme.2025.109231_bb0240","doi-asserted-by":"crossref","DOI":"10.1002\/rcm.9721","article-title":"Mass spectrometry imaging protocol for spatial mapping of lipids, N-glycans and peptides in murine lung tissue","volume":"38","author":"Ngai","year":"2024","journal-title":"Rapid Commun. Mass Spectrom."},{"issue":"W1","key":"10.1016\/j.ymgme.2025.109231_bb0245","doi-asserted-by":"crossref","first-page":"W398","DOI":"10.1093\/nar\/gkae253","article-title":"MetaboAnalyst 6.0: towards a unified platform for metabolomics data processing, analysis and interpretation","volume":"52","author":"Pang","year":"2024","journal-title":"Nucleic Acids Res."}],"container-title":["Molecular Genetics and Metabolism"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1096719225002227?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1096719225002227?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,10,25]],"date-time":"2025-10-25T23:01:03Z","timestamp":1761433263000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S1096719225002227"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,9]]},"references-count":49,"journal-issue":{"issue":"1-2","published-print":{"date-parts":[[2025,9]]}},"alternative-id":["S1096719225002227"],"URL":"https:\/\/doi.org\/10.1016\/j.ymgme.2025.109231","relation":{},"ISSN":["1096-7192"],"issn-type":[{"value":"1096-7192","type":"print"}],"subject":[],"published":{"date-parts":[[2025,9]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Defining lung pathogenesis in a murine model of mucopolysaccharidosis Type I by proteomic analysis","name":"articletitle","label":"Article Title"},{"value":"Molecular Genetics and Metabolism","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.ymgme.2025.109231","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2025 The Authors. Published by Elsevier Inc.","name":"copyright","label":"Copyright"}],"article-number":"109231"}}