{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2023,10,24]],"date-time":"2023-10-24T15:35:19Z","timestamp":1698161719950},"reference-count":19,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2005,12,5]],"date-time":"2005-12-05T00:00:00Z","timestamp":1133740800000},"content-version":"vor","delay-in-days":5209,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Prenatal Diagnosis"],"published-print":{"date-parts":[[1991,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>The de\u2010O\u2010sulphation of \u03b1\u2010linked glucosamine\u20106\u2010sulphate residues in heparan sulphate requires a specific sulphatase, glucosamine\u20106\u2010sulphatase, which has been shown to be deficient in tissues of Sanfilippo D, or mucopolysaccharidosis type IIID (MPS IIID), patients. MPS IIID fibroblasts cultured in Basal Eagle's medium supplemented with either fetal calf serum or heat\u2010inactivated fetal calf serum, MDCB or Ultraserg media had residual glucosarnine\u20106\u2010sulphatase activities towards a heparin\u2010derived trisaccharide substrate, <jats:italic>O<\/jats:italic>\u2010(\u03b1\u2010<jats:italic>N<\/jats:italic>\u2010acetylglucosamine\u20106\u2010sulphate)\u2010(1\u21924)\u2010<jats:sc>L<\/jats:sc>\u2010<jats:italic>O<\/jats:italic>\u2010(\u03b1\u2010iduronic acid\u20102\u2010sulphate)\u2010(1\u21924)\u2010<jats:sc>D<\/jats:sc>\u2010<jats:italic>O<\/jats:italic>\u20102,5\u2010anhydro [1\u2010<jats:sup>3<\/jats:sup>H]mannitol\u20106\u2010sulphate, GlcNAc6S\u2010IdoA2S\u2010anM6S, which were less than 1 per cent of the normal range for fibroblasts cultured in Basal Eagle's medium supplemented with fetal calf serum. However, the glucosamine\u20106\u2010sulphatase activities of MPS IIID fibroblasts grown in Chang's medium were similar to the activities in normal control fibroblasts which were cultured in Basal Eagle's medium. These results indicate that caution is required for prenatal diagnosis of MPS IIID patients using chorionic villi or amniotic cells cultured in Chang's medium.<\/jats:p>","DOI":"10.1002\/pd.1970110908","type":"journal-article","created":{"date-parts":[[2007,3,4]],"date-time":"2007-03-04T05:37:29Z","timestamp":1172986649000},"page":"711-717","source":"Crossref","is-referenced-by-count":5,"title":["Sanfilippo D syndrome: Correction of glucosamine\u20106\u2010sulphatase deficiency following fibroblast culture in chang's media"],"prefix":"10.1002","volume":"11","author":[{"given":"Craig","family":"Freeman","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"John J.","family":"Hopwood","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2005,12,5]]},"reference":[{"key":"e_1_2_1_2_1","doi-asserted-by":"publisher","DOI":"10.1042\/bj2710075"},{"key":"e_1_2_1_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/j.1460-2075.1989.tb03426.x"},{"key":"e_1_2_1_4_1","doi-asserted-by":"publisher","DOI":"10.1038\/icb.1977.19"},{"key":"e_1_2_1_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/0003-2697(84)90789-9"},{"key":"e_1_2_1_6_1","doi-asserted-by":"publisher","DOI":"10.1042\/bj2340083"},{"key":"e_1_2_1_7_1","doi-asserted-by":"publisher","DOI":"10.1042\/bj2460355"},{"key":"e_1_2_1_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/0003-2697(89)90303-5"},{"key":"e_1_2_1_9_1","doi-asserted-by":"publisher","DOI":"10.1042\/bj2590209"},{"key":"e_1_2_1_10_1","doi-asserted-by":"crossref","unstructured":"Freeman C. 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