{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,12]],"date-time":"2026-10-12T21:02:25Z","timestamp":1791838945703,"version":"4.3.4"},"reference-count":21,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2013,6,21]],"date-time":"2013-06-21T00:00:00Z","timestamp":1371772800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Int J Experimental Path"],"published-print":{"date-parts":[[2013,10]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Mucopolysaccharidoses (<jats:styled-content style=\"fixed-case\">MPS<\/jats:styled-content>) are lysosomal storage disorders characterized by mutations in enzymes that degrade glycosaminoglycans (<jats:styled-content style=\"fixed-case\">GAG<\/jats:styled-content>s). Joint disease is present in most forms of <jats:styled-content style=\"fixed-case\">MPS<\/jats:styled-content>, including <jats:styled-content style=\"fixed-case\">MPS<\/jats:styled-content> I. This work aimed to describe the joint disease progression in the murine model of <jats:styled-content style=\"fixed-case\">MPS<\/jats:styled-content> I. Normal (wild\u2010type) and <jats:styled-content style=\"fixed-case\">MPS<\/jats:styled-content> I mice were sacrificed at different time points (from 2 to 12\u00a0months). The knee joints were collected, and haematoxylin\u2013eosin staining was used to evaluate the articular architecture. Safranin\u2010O and <jats:styled-content style=\"fixed-case\">S<\/jats:styled-content>irius <jats:styled-content style=\"fixed-case\">R<\/jats:styled-content>ed staining was used to analyse the proteoglycan and collagen content. Additionally, we analysed the expression of the matrix\u2010degrading metalloproteinases (<jats:styled-content style=\"fixed-case\">MMP<\/jats:styled-content>s), <jats:styled-content style=\"fixed-case\">MMP<\/jats:styled-content>\u20102 and <jats:styled-content style=\"fixed-case\">MMP<\/jats:styled-content>\u20109, using immunohistochemistry. We observed progressive joint alterations from 6\u00a0months, including the presence of synovial inflammatory infiltrate, the destruction and thickening of the cartilage extracellular matrix, as well as proteoglycan and collagen depletion. Furthermore, we observed an increase in the expression of <jats:styled-content style=\"fixed-case\">MMP<\/jats:styled-content>\u20102 and <jats:styled-content style=\"fixed-case\">MMP<\/jats:styled-content>\u20109, which could conceivably explain the degenerative changes. Our results suggest that the joint disease in <jats:styled-content style=\"fixed-case\">MPS<\/jats:styled-content> I mice may be caused by a degenerative process due to increase in proteases expression, leading to loss of collagen and proteoglycans. These results may guide the development of ancillary therapies for joint disease in <jats:styled-content style=\"fixed-case\">MPS<\/jats:styled-content> I.<\/jats:p>","DOI":"10.1111\/iep.12033","type":"journal-article","created":{"date-parts":[[2013,6,21]],"date-time":"2013-06-21T05:18:47Z","timestamp":1371791927000},"page":"305-311","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":22,"title":["Characterization of joint disease in mucopolysaccharidosis type I mice"],"prefix":"10.1111","volume":"94","author":[{"given":"Patricia G.","family":"de Oliveira","sequence":"first","affiliation":[{"name":"Programa de p\u00f3s\u2010gradua\u00e7\u00e3o em medicina: ci\u00eancias medicas Universidade Federal do Rio Grande do Sul Rio Grande do Sul Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Guilherme","family":"Baldo","sequence":"additional","affiliation":[{"name":"Programa de p\u00f3s\u2010gradua\u00e7\u00e3o em Ci\u00eancias Biol\u00f3gicas: Bioqu\u00edmica Universidade Federal do Rio Grande do Sul Rio Grande do Sul Brazil"},{"name":"Centro de Terapia G\u00eanica Hospital de Clinicas e Porto Alegre Porto Alegre Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fabiana Q.","family":"Mayer","sequence":"additional","affiliation":[{"name":"Centro de Terapia G\u00eanica Hospital de Clinicas e Porto Alegre Porto Alegre Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Barbara","family":"Martinelli","sequence":"additional","affiliation":[{"name":"Centro de Terapia G\u00eanica Hospital de Clinicas e Porto Alegre Porto Alegre Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luise","family":"Meurer","sequence":"additional","affiliation":[{"name":"Servi\u00e7o de Patologia Hospital de Clinicas de Porto Alegre Porto Alegre Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roberto","family":"Giugliani","sequence":"additional","affiliation":[{"name":"Programa de p\u00f3s\u2010gradua\u00e7\u00e3o em Ci\u00eancias Biol\u00f3gicas: Bioqu\u00edmica Universidade Federal do Rio Grande do Sul Rio Grande do Sul Brazil"},{"name":"Centro de Terapia G\u00eanica Hospital de Clinicas e Porto Alegre Porto Alegre Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ursula","family":"Matte","sequence":"additional","affiliation":[{"name":"Centro de Terapia G\u00eanica Hospital de Clinicas e Porto Alegre Porto Alegre Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ricardo M.","family":"Xavier","sequence":"additional","affiliation":[{"name":"Programa de p\u00f3s\u2010gradua\u00e7\u00e3o em medicina: ci\u00eancias medicas Universidade Federal do Rio Grande do Sul Rio Grande do Sul Brazil"},{"name":"Servico de Reumatologia Hospital de Clinicas de Porto Alegre Porto Alegre Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2013,6,21]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2011.08.018"},{"key":"e_1_2_10_3_1","first-page":"1","article-title":"Bone marrow cells reduce collagen deposition in the rat model of common bile duct ligation","volume":"2","author":"Baldo G.","year":"2011","journal-title":"Cell. Sci. Ther."},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbr.2012.04.051"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/6.4.503"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1590\/S0104-42302010000300009"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0002-9440(10)64961-9"},{"key":"e_1_2_10_8_1","first-page":"131","article-title":"Expression profiling of metalloproteinases and inhibitors in cartilage","volume":"86","author":"Kevorkian L.","year":"2005","journal-title":"Int. J. Clin. Exp. Pathol."},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2008.03.018"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddn088"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2009.03.005"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.252784899"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.bor.0000147283.40529.13"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1399-0004.1998.tb02745.x"},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.joca.2010.05.026"},{"key":"e_1_2_10_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0955-0674(98)80035-5"},{"key":"e_1_2_10_17_1","doi-asserted-by":"publisher","DOI":"10.1203\/01.PDR.0000156510.96253.5A"},{"key":"e_1_2_10_18_1","doi-asserted-by":"publisher","DOI":"10.2353\/ajpath.2008.070564"},{"key":"e_1_2_10_19_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-010-9059-9"},{"key":"e_1_2_10_20_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2010-04-278234"},{"key":"e_1_2_10_21_1","doi-asserted-by":"publisher","DOI":"10.2353\/ajpath.2009.090211"},{"key":"e_1_2_10_22_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.23654"}],"container-title":["International Journal of Experimental Pathology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fiep.12033","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/iep.12033","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,3]],"date-time":"2023-10-03T18:39:43Z","timestamp":1696358383000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/iep.12033"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2013,6,21]]},"references-count":21,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2013,10]]}},"alternative-id":["10.1111\/iep.12033"],"URL":"https:\/\/doi.org\/10.1111\/iep.12033","archive":["Portico"],"relation":{},"ISSN":["0959-9673","1365-2613"],"issn-type":[{"value":"0959-9673","type":"print"},{"value":"1365-2613","type":"electronic"}],"subject":[],"published":{"date-parts":[[2013,6,21]]},"assertion":[{"value":"2013-01-17","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2013-05-11","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2013-06-21","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}