{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,1]],"date-time":"2025-11-01T13:32:03Z","timestamp":1762003923440},"reference-count":7,"publisher":"Oxford University Press (OUP)","issue":"1","license":[{"start":{"date-parts":[[2007,1,1]],"date-time":"2007-01-01T00:00:00Z","timestamp":1167609600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2007,1,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Background: A treatment for mucopolysaccharidosis II (Hunter syndrome) has recently become available. Therefore, we developed a high-throughput assay method appropriate for newborn screening for the relevant enzyme, iduronate 2-sulfatase.<\/jats:p>\n               <jats:p>Methods: We synthesized a new iduronate 2-sulfatase substrate that can be used to assay the enzyme by use of tandem mass spectrometry together with an internal standard. The assay uses a dried blood spot on a newborn screening card as the enzyme source.<\/jats:p>\n               <jats:p>Results: When the assay was tested on dried blood spots, the iduronate 2-sulfatase activity measured for 13 patients with Hunter syndrome was well below the interval found for 57 randomly chosen newborns. The assay was more sensitive than previously reported iduronate 2-sulfatase assays.<\/jats:p>\n               <jats:p>Conclusions: This newly developed tandem mass spectrometry assay has the potential to be adopted for newborn screening of Hunter syndrome. This method also has the potential to be carried out in multiplex fashion to assay several different enzymes relevant to lysosomal storage diseases that are assayed in a single infusion into the mass spectrometer.<\/jats:p>","DOI":"10.1373\/clinchem.2006.077263","type":"journal-article","created":{"date-parts":[[2006,11,3]],"date-time":"2006-11-03T01:34:47Z","timestamp":1162517687000},"page":"137-140","source":"Crossref","is-referenced-by-count":53,"title":["Tandem Mass Spectrometry for the Direct Assay of Enzymes in Dried Blood Spots: Application to Newborn Screening for Mucopolysaccharidosis II (Hunter Disease)"],"prefix":"10.1093","volume":"53","author":[{"given":"Ding","family":"Wang","sequence":"first","affiliation":[{"name":"Departments of Chemistry, University of Washington, Seattle, Washington"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tim","family":"Wood","sequence":"first","affiliation":[{"name":"Biochemical Genetics Laboratory, Greenwood Genetic Center, Greenwood, South Carolina"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Martin","family":"Sadilek","sequence":"first","affiliation":[{"name":"Departments of Chemistry, University of Washington, Seattle, Washington"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"C Ronald","family":"Scott","sequence":"first","affiliation":[{"name":"Departments of Pediatrics, University of Washington, Seattle, Washington"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Frantisek","family":"Turecek","sequence":"first","affiliation":[{"name":"Departments of Chemistry, University of Washington, Seattle, Washington"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael H","family":"Gelb","sequence":"first","affiliation":[{"name":"Departments of Chemistry, University of Washington, Seattle, Washington"},{"name":"Departments of Biochemistry, University of Washington, Seattle, Washington"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"286","published-online":{"date-parts":[[2007,1,1]]},"reference":[{"key":"2020022802442201100_R1","doi-asserted-by":"crossref","unstructured":"Chamoles NA, Blanco M, Gaggioli D, Casentini C. Tay-Sachs and Sandhoff diseases: enzymatic diagnosis in dried blood spots on filter paper: retrospective diagnoses in newborn-screening cards. Clin Chim Acta2002;318:133-137.","DOI":"10.1016\/S0009-8981(02)00002-5"},{"key":"2020022802442201100_R2","doi-asserted-by":"crossref","unstructured":"Li Y, Brockman K, Turecek F, Scott CR, Gelb MH. Tandem mass spectrometry for the direct assay of enzymes in dried blood spots: application to newborn screening for Krabbe disease. Clin Chem2004;50:638-640.","DOI":"10.1373\/clinchem.2003.028381"},{"key":"2020022802442201100_R3","doi-asserted-by":"crossref","unstructured":"Li Y, Scott CR, Chamoles NA, Ghavami A, Pinto BM, Turecek F, et al. Direct multiplex assay of lysosomal enzymes in dried blood spots for newborn screening. Clin Chem2004;50:1785-1796.","DOI":"10.1373\/clinchem.2004.035907"},{"key":"2020022802442201100_R4","doi-asserted-by":"crossref","unstructured":"Wang D, Eadala B, Sadilek M, Chamoles NA, Turecek F, Scott CR, et al. Tandem mass spectrometric analysis of dried blood spots for screening of mucopolysaccharidosis I in newborns. Clin Chem2005;51:898-900.","DOI":"10.1373\/clinchem.2004.047167"},{"key":"2020022802442201100_R5","doi-asserted-by":"crossref","unstructured":"Muenzer J, Lamsa JC, Garcia A, Dacosta J, Garcia J, Treco DA. Enzyme replacement therapy in mucopolysaccharidosis type II (Hunter syndrome): a preliminary report. Acta Paediatr Suppl2002;91:98-99.","DOI":"10.1111\/j.1651-2227.2002.tb03118.x"},{"key":"2020022802442201100_R6","doi-asserted-by":"crossref","unstructured":"Hopwood JJ. \u03b1-L-Iduronidase, \u03b2-D-glucuronidase, and 2-sulfo-L-iduronate 2-sulfatase: preparation and characterization of radioactive substrates from heparin. Carbohydr Res1979;69:203-216.","DOI":"10.1016\/S0008-6215(00)85765-1"},{"key":"2020022802442201100_R7","doi-asserted-by":"crossref","unstructured":"Keulemans JLM, Sinigerska I, Garritsen VH, Huijmans JGM, Voznyi YV, van Diggelen OP, et al. Prenatal diagnosis of the Hunter syndrome and the introduction of a new fluorimetric enzyme assay. Prenat Diagn2002;22:1016-1021.","DOI":"10.1002\/pd.457"}],"container-title":["Clinical Chemistry"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/academic.oup.com\/clinchem\/article-pdf\/53\/1\/137\/32690229\/clinchem0137.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"http:\/\/academic.oup.com\/clinchem\/article-pdf\/53\/1\/137\/32690229\/clinchem0137.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2020,2,28]],"date-time":"2020-02-28T07:46:07Z","timestamp":1582875967000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/clinchem\/article\/53\/1\/137\/5627181"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2007,1,1]]},"references-count":7,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2007,1,1]]},"published-print":{"date-parts":[[2007,1,1]]}},"URL":"https:\/\/doi.org\/10.1373\/clinchem.2006.077263","relation":{},"ISSN":["0009-9147","1530-8561"],"issn-type":[{"value":"0009-9147","type":"print"},{"value":"1530-8561","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2007,1,1]]},"published":{"date-parts":[[2007,1,1]]}}}