{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2023,11,21]],"date-time":"2023-11-21T05:32:50Z","timestamp":1700544770774},"reference-count":17,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[1983,6,1]],"date-time":"1983-06-01T00:00:00Z","timestamp":423273600000},"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":[[1983,6]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>The substrate<jats:italic>O<\/jats:italic>\u2010(<jats:italic>\u03b1<\/jats:italic>\u2010<jats:sc>L<\/jats:sc>\u2010idopyranosyuronic acid\u20102\u2010sulphate)\u2010(1 \u2192 4)\u20102,5\u2010anhydro\u2010<jats:sc>D<\/jats:sc>\u2010(<jats:sup>3<\/jats:sup>H\u20101) mannitol\u20106\u2010sulphate was used at a final concentration of 50 mmol\/l to measure the<jats:italic>\u03b1<\/jats:italic>\u2010<jats:sc>L<\/jats:sc>\u2010idurono\u20102\u2010sulphate sulphatase activities of cell extracts, serum and amniotic fluid. Activities were measured after dialysis against water, to avoid the inhibitory effect of sodium chloride and the reaction products separated by ion\u2010exchange chromatography on ECTEOLA cellulose. The enzyme present in normal serum had an apparent<jats:italic>K<\/jats:italic><jats:sub>m<\/jats:sub> of 0.12 mmol\/l. The mean activities of normal serum, fibroblasts and leukocytes were 0.61, 16.63 and 18.75 nmol\/mg protein per hour respectively, while corresponding \u2018Hunter\u2019 tissues contained between 1% and 3% of normal activity. Cultured normal amniotic cells had a mean activity of 8.46 nmol\/mg protein per hour, while those cultured from \u2018Hunter\u2019 patients contained about 6% of normal activity. Hunter disease can thus be readily diagnosed in a wide variety of tissues. Samples from three obligate heterozygotes were found to have iduronate sulphatase activities amounting to 41%, 57% and 63% of normal values.<\/jats:p>","DOI":"10.1007\/bf01800738","type":"journal-article","created":{"date-parts":[[2005,6,21]],"date-time":"2005-06-21T16:50:02Z","timestamp":1119372602000},"page":"108-111","source":"Crossref","is-referenced-by-count":3,"title":["The iduronate sulphatase activities of cells and tissue fluids from patients with Hunter syndrome and normal controls"],"prefix":"10.1002","volume":"6","author":[{"given":"M. F.","family":"Dean","sequence":"first","affiliation":[{"name":"Kennedy Institute of Rheumatology  6 Bute Gardens, Hammersmith London W6 7DW UK"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[1983,6]]},"reference":[{"key":"e_1_2_1_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/0009-8981(81)90274-6"},{"key":"e_1_2_1_3_1","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.70.7.2134"},{"key":"e_1_2_1_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/0003-2697(62)90095-7"},{"key":"e_1_2_1_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1399-0004.1978.tb02109.x"},{"key":"e_1_2_1_6_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF01801722"},{"key":"e_1_2_1_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/0006-2944(77)90011-4"},{"key":"e_1_2_1_8_1","first-page":"199","article-title":"Automated procedures for the determination of proteins, hexose and uronic acid in column effluents","volume":"4","author":"Heineg\u00e5rd D.","year":"1973","journal-title":"Chem. 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Genet."}],"container-title":["Journal of Inherited Metabolic Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1007\/BF01800738","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1007\/BF01800738","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,16]],"date-time":"2023-09-16T05:37:39Z","timestamp":1694842659000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1007\/BF01800738"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1983,6]]},"references-count":17,"journal-issue":{"issue":"3","published-print":{"date-parts":[[1983,6]]}},"alternative-id":["10.1007\/BF01800738"],"URL":"https:\/\/doi.org\/10.1007\/bf01800738","archive":["Portico"],"relation":{},"ISSN":["0141-8955","1573-2665"],"issn-type":[{"value":"0141-8955","type":"print"},{"value":"1573-2665","type":"electronic"}],"subject":[],"published":{"date-parts":[[1983,6]]}}}