{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2024,1,27]],"date-time":"2024-01-27T03:38:03Z","timestamp":1706326683827},"reference-count":19,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[1989,4,20]],"date-time":"1989-04-20T00:00:00Z","timestamp":609033600000},"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":[[1990,1]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>The low abundance lysosomal enzyme<jats:italic>N<\/jats:italic>\u2010acetylgalactosamine 4\u2010sulphatase (4\u2010sulphatase) has been quantified using a microimmunopurification step and a monoclonal\u2010based ELISA detection system. The assay is similar in principle to a two\u2010site ELISA but uses a single monoclonal antibody against one epitope to bind 4\u2010sulphatase in two separate assay steps. The sensitivity of this assay is sufficient to allow the quantification of 4\u2010sulphatase in human cultured skin fibroblasts derived from normal controls and patients deficient in 4\u2010sulphatase activity (mucopolysaccharidosis type VI or Maroteaux\u2010Lamy syndrome). The results obtained suggest a range of mucopolysaccharidosis type VI or 4\u2010sulphatase deficient mutants, from those expressing little or no quantifiable 4\u2010sulphatase protein to those examples with quantifiable levels of 4\u2010sulphatase protein which is enzymically inactive. Phenotypic variability in patients with a 4\u2010sulphatase deficiency may therefore be partially attributed to a range of protein expressions. The method should allow the determination of 4\u2010sulphatase specific activity in mucopolysaccharidosis type VI patients.<\/jats:p>","DOI":"10.1007\/bf01799338","type":"journal-article","created":{"date-parts":[[2005,6,21]],"date-time":"2005-06-21T13:55:43Z","timestamp":1119362143000},"page":"108-120","source":"Crossref","is-referenced-by-count":8,"title":["Immunoquantification of the low abundance lysosomal enzyme<i>N<\/i>\u2010acetylgalactosamine 4\u2010sulphatase"],"prefix":"10.1002","volume":"13","author":[{"given":"D. A.","family":"Brooks","sequence":"first","affiliation":[{"name":"Lysosomal Diseases Research Unit, Department of Chemical Pathology Adelaide Childrens Hospital North Adelaide SA 5006 Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"P. A. G.","family":"McCourt","sequence":"additional","affiliation":[{"name":"Lysosomal Diseases Research Unit, Department of Chemical Pathology Adelaide Childrens Hospital North Adelaide SA 5006 Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"G. J.","family":"Gibson","sequence":"additional","affiliation":[{"name":"Lysosomal Diseases Research Unit, Department of Chemical Pathology Adelaide Childrens Hospital North Adelaide SA 5006 Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J. 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