{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T15:28:57Z","timestamp":1791300537669,"version":"4.1.0"},"reference-count":0,"publisher":"National Academy of Sciences","issue":"7","content-domain":{"domain":["www.pnas.org"],"crossmark-restriction":true},"short-container-title":["Proc. Natl. Acad. Sci. U.S.A."],"published-print":{"date-parts":[[1973,7]]},"abstract":"<jats:p>\n                    Skin fibroblasts cultured from patients affected with the Hunter syndrome are deficient in the activity of a protein, named the \u201cHunter corrective factor,\u201d that is required for degradation of dermatan and heparan sulfates. We now show that this factor, purified from human urine, removes about 2% of the sulfate residues from [\n                    <jats:sup>35<\/jats:sup>\n                    S]mucopolysaccharide accumulated within Hunter fibroblasts; these groups are derived from \u201coversulfated\u201d regions of the polymer. Acetone-powder extracts of fibroblasts derived from patients with the Hunter syndrome are deficient in this sulfatase, in contrast to similar extracts from fibroblasts of individuals of other genotype. Hunter corrective factor coupled to \u03b1-L-iduronidase (or alternatively, mixed extracts from Hurler and Hunter fibroblasts) release iduronic acid from 4-\n                    <jats:italic>O<\/jats:italic>\n                    -\u03b1-L-sulfoiduronosyl-D-sulfoanhydromannose. We conclude that the Hunter corrective factor is a sulfatase for sulfated iduronic acid residues.\n                  <\/jats:p>","DOI":"10.1073\/pnas.70.7.2134","type":"journal-article","created":{"date-parts":[[2006,5,31]],"date-time":"2006-05-31T02:40:11Z","timestamp":1149043211000},"page":"2134-2138","update-policy":"https:\/\/doi.org\/10.1073\/pnas.cm10313","source":"Crossref","is-referenced-by-count":173,"title":["The Defect in the Hunter Syndrome: Deficiency of Sulfoiduronate Sulfatase"],"prefix":"10.1073","volume":"70","author":[{"given":"Gideon","family":"Bach","sequence":"first","affiliation":[{"name":"National Institute of Arthritis, Metabolism, and Digestive Diseases, National Institutes of Health, Bethesda, Maryland 20014"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"suffix":"Jr.","given":"Frank","family":"Eisenberg","sequence":"additional","affiliation":[{"name":"National Institute of Arthritis, Metabolism, and Digestive Diseases, National Institutes of Health, Bethesda, Maryland 20014"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Cantz","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, University of Kiel, Kiel, Federal Republic of Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elizabeth F.","family":"Neufeld","sequence":"additional","affiliation":[{"name":"National Institute of Arthritis, Metabolism, and Digestive Diseases, National Institutes of Health, Bethesda, Maryland 20014"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"341","published-online":{"date-parts":[[1973,7]]},"container-title":["Proceedings of the National Academy of Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/pnas.org\/doi\/pdf\/10.1073\/pnas.70.7.2134","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,4,13]],"date-time":"2022-04-13T10:44:29Z","timestamp":1649846669000},"score":1,"resource":{"primary":{"URL":"https:\/\/pnas.org\/doi\/full\/10.1073\/pnas.70.7.2134"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1973,7]]},"references-count":0,"journal-issue":{"issue":"7","published-print":{"date-parts":[[1973,7]]}},"alternative-id":["10.1073\/pnas.70.7.2134"],"URL":"https:\/\/doi.org\/10.1073\/pnas.70.7.2134","relation":{},"ISSN":["0027-8424","1091-6490"],"issn-type":[{"value":"0027-8424","type":"print"},{"value":"1091-6490","type":"electronic"}],"subject":[],"published":{"date-parts":[[1973,7]]},"assertion":[{"value":"1973-07-01","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}