{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,1]],"date-time":"2026-08-01T15:59:26Z","timestamp":1785599966099,"version":"3.56.0"},"reference-count":29,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2012,9,13]],"date-time":"2012-09-13T00:00:00Z","timestamp":1347494400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[2013,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Mucopolysaccharidoses (MPS) are a group of lysosomal storage diseases caused by mutations in lysosomal enzymes involved in degradation of glycosaminoglycans (GAGs). Patients with MPS grow poorly and become physically disabled due to systemic bone disease. While many of the major skeletal effects in mouse models for MPS have been described, no detailed analysis that compares GAGs levels and characteristics of bone by micro\u2010CT has been done. The aims of this study were to assess severity of bone dysplasia among four MPS mouse models (MPS I, IIIA, IVA and VII), to determine the relationship between severity of bone dysplasia and serum keratan sulfate (KS) and heparan sulfate (HS) levels in those models, and to explore the mechanism of KS elevation in MPS I, IIIA, and VII mouse models. Clinically, MPS VII mice had the most severe bone pathology; however, MPS I and IVA mice also showed skeletal pathology. MPS I and VII mice showed severe bone dysplasia, higher bone mineral density, narrowed spinal canal, and shorter sclerotic bones by micro\u2010CT and radiographs. Serum KS and HS levels were elevated in MPS I, IIIA, and VII mice. Severity of skeletal disease displayed by micro\u2010CT, radiographs and histopathology correlated with the level of KS elevation. We showed that elevated HS levels in MPS mouse models could inhibit N\u2010acetylgalactosamine\u20106\u2010sulfate sulfatase enzyme. These studies suggest that KS could be released from chondrocytes affected by accumulation of other GAGs and that KS could be useful as a biomarker for severity of bone dysplasia in MPS disorders.<\/jats:p>","DOI":"10.1007\/s10545-012-9522-x","type":"journal-article","created":{"date-parts":[[2012,9,12]],"date-time":"2012-09-12T08:51:23Z","timestamp":1347439883000},"page":"235-246","source":"Crossref","is-referenced-by-count":63,"title":["Assessment of bone dysplasia by micro\u2010CT and glycosaminoglycan levels in mouse models for mucopolysaccharidosis type I, IIIA, IVA, and VII"],"prefix":"10.1002","volume":"36","author":[{"given":"Daniel J.","family":"Rowan","sequence":"first","affiliation":[{"name":"School of Medicine Saint Louis University St. Louis MO USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shunji","family":"Tomatsu","sequence":"additional","affiliation":[{"name":"Department of Biomedical Research Alfred I. duPont Institute Hospital for Children Wilmington DE USA"},{"name":"Department of Orthopedic Surgery Alfred I. duPont Institute Hospital for Children Wilmington DE USA"},{"name":"Skeletal Dysplasia Lab Pediatric Orthopedic Surgery Nemours\/Alfred I. duPont Hospital for Children 1600 Rockland Rd. Wilmington DE 19899\u20100269 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeffrey H.","family":"Grubb","sequence":"additional","affiliation":[{"name":"Edward A. Doisy Department of Biochemistry and Molecular Biology Saint Louis University St. Louis MO USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adriana M.","family":"Monta\u00f1o","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Saint Louis University St. Louis MO USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"William S.","family":"Sly","sequence":"additional","affiliation":[{"name":"Edward A. Doisy Department of Biochemistry and Molecular Biology Saint Louis University St. Louis MO USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2012,9,13]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1136\/ard.2008.095315"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1093\/glycob\/11.1.99"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1093\/glycob\/9.12.1389"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1172\/JCI114010"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/6.4.503"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.271.47.30136"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddm223"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1063-4584(05)80042-1"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-007-0641-8"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1006\/cyto.2002.2002"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.0803715105"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1651-2227.2007.00211.x"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1042\/bj3340297"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1002\/jgm.1229"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10059-010-0083-2"},{"key":"e_1_2_9_17_1","doi-asserted-by":"publisher","DOI":"10.1242\/dev.01948"},{"key":"e_1_2_9_18_1","doi-asserted-by":"publisher","DOI":"10.1161\/01.RES.81.5.656"},{"key":"e_1_2_9_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bone.2008.04.009"},{"key":"e_1_2_9_20_1","first-page":"3421","volume-title":"The metabolic and molecular bases of inherited disease","author":"Neufeld EF","year":"2001"},{"key":"e_1_2_9_21_1","doi-asserted-by":"publisher","DOI":"10.1523\/JNEUROSCI.4127-05.2006"},{"key":"e_1_2_9_22_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.274.45.32507"},{"key":"e_1_2_9_23_1","doi-asserted-by":"publisher","DOI":"10.1359\/JBMR.050209"},{"key":"e_1_2_9_24_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.051623698"},{"key":"e_1_2_9_25_1","doi-asserted-by":"publisher","DOI":"10.1038\/359076a0"},{"key":"e_1_2_9_26_1","doi-asserted-by":"publisher","DOI":"10.1093\/hmg\/ddg366"},{"key":"e_1_2_9_27_1","first-page":"3321","article-title":"Development of MPS IVA mouse","volume":"14","author":"Tomatsu S","year":"2005","journal-title":"Genet"},{"key":"e_1_2_9_28_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-005-5673-3"},{"key":"e_1_2_9_29_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2007.02.009"},{"key":"e_1_2_9_30_1","doi-asserted-by":"publisher","DOI":"10.1038\/mt.2010.32"}],"container-title":["Journal of Inherited Metabolic Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1007\/s10545-012-9522-x","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,15]],"date-time":"2023-09-15T08:00:28Z","timestamp":1694764828000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1007\/s10545-012-9522-x"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2012,9,13]]},"references-count":29,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2013,3]]}},"alternative-id":["10.1007\/s10545-012-9522-x"],"URL":"https:\/\/doi.org\/10.1007\/s10545-012-9522-x","archive":["Portico"],"relation":{},"ISSN":["0141-8955","1573-2665"],"issn-type":[{"value":"0141-8955","type":"print"},{"value":"1573-2665","type":"electronic"}],"subject":[],"published":{"date-parts":[[2012,9,13]]}}}