{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T02:09:14Z","timestamp":1790820554731,"version":"4.1.0"},"reference-count":18,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2007,9,16]],"date-time":"2007-09-16T00:00:00Z","timestamp":1189900800000},"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":[[2007,11]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Mucopolysaccharidosis II (MPS II, Hunter syndrome in humans) is an X\u2010linked inherited lysosomal storage disease caused by a deficiency in the lysosomal enzyme iduronate\u20102\u2010sulfatase (I2S). I2S catalyses a step in the catabolism of glycosaminoglycans (GAGs) dermatan sulfate and heparan sulfate, and when it is deficient or absent GAGs accumulate in tissues and organs. Male knockout mice (IdS\u2010KO), which lack the gene coding for I2S, exhibit many of the characteristics seen in the human disease. Compared to wild\u2010type control mice, urine GAG excretion was elevated at 4 weeks of age and remained high throughout the lifespan, and tissue GAG levels were elevated as early as 7 weeks of age. Liver, spleen and other organs were significantly larger in the IdS\u2010KO mice than in the wild\u2010type. Radiographic examination revealed sclerosis and enlargement of the skull at 4 weeks of age and appendicular bone enlargement at 10\u201313 weeks of age. Micro CT scans showed severe periosteal bone formation at the lateral aspect of the distal tibia and calcification of the calcaneus tendon. This model was used in the development of idursulfase for treatment of MPS II and may continue to be useful in the evaluation of treatment strategies of this chronic and progressive disorder.<\/jats:p>","DOI":"10.1007\/s10545-007-0641-8","type":"journal-article","created":{"date-parts":[[2007,9,17]],"date-time":"2007-09-17T07:57:39Z","timestamp":1190015859000},"page":"924-934","source":"Crossref","is-referenced-by-count":75,"title":["The characterization of a murine model of mucopolysaccharidosis II (Hunter syndrome)"],"prefix":"10.1002","volume":"30","author":[{"given":"A. R.","family":"Garcia","sequence":"first","affiliation":[{"name":"Shire Human Genetic Therapies, Inc. Preclinical Research 700 Main Street Cambridge MA 02039 USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J.","family":"Pan","sequence":"additional","affiliation":[{"name":"Shire Human Genetic Therapies, Inc. 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