{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T15:29:47Z","timestamp":1791300587622,"version":"4.1.0"},"reference-count":0,"publisher":"National Academy of Sciences","issue":"8","content-domain":{"domain":["www.pnas.org"],"crossmark-restriction":true},"short-container-title":["Proc. Natl. Acad. Sci. U.S.A."],"published-print":{"date-parts":[[1972,8]]},"abstract":"<jats:p>Skin fibroblasts cultured from patients affected with the Hurler or Scheie syndromes (mucopoly-saccharidoses I or V, respectively) have a functional deficiency of a protein required for catabolism of sulfated mucopolysaccharide that has been designated the \u201cHurler corrective factor.\u201d We now show Hurler factor purified from normal human urine to be associated with \u03b1-L-iduronidase activity. Cell lines deficient in Hurler corrective factor have no detectable activity of \u03b1-L-iduronidase (less than 3% of that found in cells from individuals of other genotypes). Such correspondence indicates that Hurler corrective factor and \u03b1-L-iduronidase are the same entity. Correction of deficient cells is accompanied by an efficient uptake of \u03b1-L-iduronidase from the medium.<\/jats:p>","DOI":"10.1073\/pnas.69.8.2048","type":"journal-article","created":{"date-parts":[[2006,5,31]],"date-time":"2006-05-31T02:37:04Z","timestamp":1149043024000},"page":"2048-2051","update-policy":"https:\/\/doi.org\/10.1073\/pnas.cm10313","source":"Crossref","is-referenced-by-count":174,"title":["The Defect in the Hurler and Scheie Syndromes: Deficiency of \u03b1-L-Iduronidase"],"prefix":"10.1073","volume":"69","author":[{"given":"Gideon","family":"Bach","sequence":"first","affiliation":[{"name":"The National Institute of Arthritis and Metabolic Diseases, National Institutes of Health, Bethesda, Maryland 20014"},{"name":"Department of Biochemistry, University of Illinois, College of Medicine, Chicago, Ill. 60612"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert","family":"Friedman","sequence":"additional","affiliation":[{"name":"The National Institute of Arthritis and Metabolic Diseases, National Institutes of Health, Bethesda, Maryland 20014"},{"name":"Department of Biochemistry, University of Illinois, College of Medicine, Chicago, Ill. 60612"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bernard","family":"Weissmann","sequence":"additional","affiliation":[{"name":"The National Institute of Arthritis and Metabolic Diseases, National Institutes of Health, Bethesda, Maryland 20014"},{"name":"Department of Biochemistry, University of Illinois, College of Medicine, Chicago, Ill. 60612"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elizabeth F.","family":"Neufeld","sequence":"additional","affiliation":[{"name":"The National Institute of Arthritis and Metabolic Diseases, National Institutes of Health, Bethesda, Maryland 20014"},{"name":"Department of Biochemistry, University of Illinois, College of Medicine, Chicago, Ill. 60612"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"341","published-online":{"date-parts":[[1972,8]]},"container-title":["Proceedings of the National Academy of Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/pnas.org\/doi\/pdf\/10.1073\/pnas.69.8.2048","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,4,13]],"date-time":"2022-04-13T10:41:27Z","timestamp":1649846487000},"score":1,"resource":{"primary":{"URL":"https:\/\/pnas.org\/doi\/full\/10.1073\/pnas.69.8.2048"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1972,8]]},"references-count":0,"journal-issue":{"issue":"8","published-print":{"date-parts":[[1972,8]]}},"alternative-id":["10.1073\/pnas.69.8.2048"],"URL":"https:\/\/doi.org\/10.1073\/pnas.69.8.2048","relation":{},"ISSN":["0027-8424","1091-6490"],"issn-type":[{"value":"0027-8424","type":"print"},{"value":"1091-6490","type":"electronic"}],"subject":[],"published":{"date-parts":[[1972,8]]},"assertion":[{"value":"1972-08-01","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}