{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,24]],"date-time":"2026-07-24T00:16:43Z","timestamp":1784852203849,"version":"3.55.0"},"reference-count":14,"publisher":"American Association for the Advancement of Science (AAAS)","issue":"4324","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Science"],"published-print":{"date-parts":[[1978,1,6]]},"abstract":"<jats:p>\n            [1-\n            <jats:sup>3<\/jats:sup>\n            H]Galactitol-6-sulfate,\n            <jats:italic>N<\/jats:italic>\n            -[1-\n            <jats:sup>3<\/jats:sup>\n            H]acetylgalactosaminitol-6-sulfate,\n            <jats:italic>N<\/jats:italic>\n            -[1-\n            <jats:sup>3<\/jats:sup>\n            H]acetylglucosaminitol-6-sulfate,\n            <jats:italic>N<\/jats:italic>\n            -acetylglucosamine-6-sulfate, and 6-sulfated tetrasaccharides from chondroitin-6-sulfate have been used for the measurementof 6-sulfatase activity of extracts of normal skin fibroblasts and of fibroblasts cultured from patients with genetic mucopolysaccharidoses.With these substrates, extracts of fibroblasts derived from Morquio patients lack or have greatly reduced activities for galactitol-6-sulfate,\n            <jats:italic>N<\/jats:italic>\n            -acetylgalactosaminitol-6-sulfate, and 6-sulfated tetrasaccharides but have normal activity for\n            <jats:italic>N<\/jats:italic>\n            -acetylglucosamine-6-sulfate and its alditol; those derived from a patient with a newly discovered mucopolysaccharidosis havegreatly reduced activity for\n            <jats:italic>N<\/jats:italic>\n            -acetylglucosamine-6-sulfate and its alditol but normal activity for galactitol-6-sulfate,\n            <jats:italic>N<\/jats:italic>\n            -acetylgalactosaminitol-6-sulfate, and the 6-sulfated tetrasaccharides. These findings demonstrate the existence of two differenthexosamine-6-sulfate sulfatases, specific for the glucose or galactose configuration of their substrates. Their respectivedeficiencies, causing inability to degrade keratan sulfate and heparan sulfate in one case and keratan sulfate and chondroitin-6-sulfatein the other, are responsible for different clinical phenotypes.\n          <\/jats:p>","DOI":"10.1126\/science.199.4324.79","type":"journal-article","created":{"date-parts":[[2006,10,5]],"date-time":"2006-10-05T16:35:25Z","timestamp":1160066125000},"page":"79-81","source":"Crossref","is-referenced-by-count":56,"title":["Deficiencies of Glucosamine-6-Sulfate or Galactosamine-6-Sulfate Sulfatases Are Responsible for Different Mucopolysaccharidoses"],"prefix":"10.1126","volume":"199","author":[{"given":"Nicola","family":"Di Ferrante","sequence":"first","affiliation":[{"name":"Laboratories of Connective Tissue Research, Department of Biochemistry, Baylor College of Medicine, Houston, Texas 77030"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Leonard C.","family":"Ginsberg","sequence":"additional","affiliation":[{"name":"Laboratories of Connective Tissue Research, Department of Biochemistry, Baylor College of Medicine, Houston, Texas 77030"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patricia V.","family":"Donnelly","sequence":"additional","affiliation":[{"name":"Laboratories of Connective Tissue Research, Department of Biochemistry, Baylor College of Medicine, Houston, Texas 77030"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniela T.","family":"Di Ferrante","sequence":"additional","affiliation":[{"name":"Laboratories of Connective Tissue Research, Department of Biochemistry, Baylor College of Medicine, Houston, Texas 77030"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C. Thomas","family":"Caskey","sequence":"additional","affiliation":[{"name":"Division of Medical Genetics, Department of Medicine, Baylor College of Medicine"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"221","reference":[{"key":"e_1_2_1_1_1","doi-asserted-by":"crossref","first-page":"6408","DOI":"10.1016\/S0021-9258(19)43461-3","volume":"248","year":"1973","unstructured":"DIETRICH, C.P., SEQUENTIAL DEGRADATION OF HEPARIN IN FLAVOBACTERIUM-HEPARINUM - PURIFICATION AND PROPERTIES OF 5 ENZYMES INVOLVED IN HEPARIN DEGRADATION, JOURNAL OF BIOLOGICAL CHEMISTRY 248: 6408 (1973).","journal-title":"JOURNAL OF BIOLOGICAL CHEMISTRY"},{"key":"e_1_2_1_2_1","doi-asserted-by":"crossref","first-page":"261","DOI":"10.1007\/978-1-4684-7735-1_18","volume":"68","year":"1976","unstructured":"DORFMAN, A, ADV EXP MED BIOL 68: 261 (1976).","journal-title":"ADV EXP MED BIOL"},{"key":"e_1_2_1_3_1","doi-asserted-by":"crossref","first-page":"80","DOI":"10.1016\/0006-2944(77)90011-4","volume":"17","year":"1977","unstructured":"GINSBERG, L.C., SENSITIVE METHODS FOR DETERMINATION OF ESTER SULFATE IN BIOLOGICAL-SYSTEMS, BIOCHEMICAL MEDICINE 17: 80 (1977).","journal-title":"BIOCHEMICAL MEDICINE"},{"key":"e_1_2_1_4_1","unstructured":"Ginsberg L. C. in preparation."},{"key":"e_1_2_1_5_1","unstructured":"GINSBERG L.C. UNPUBLISHED RESULTS."},{"key":"e_1_2_1_6_1","first-page":"68","volume":"7","year":"1976","unstructured":"HORTON, D, METHODS IN CARBOHYDRATE CHEMISTRY 7: 68 (1976).","journal-title":"METHODS IN CARBOHYDRATE CHEMISTRY"},{"key":"e_1_2_1_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0021-9258(19)52451-6"},{"key":"e_1_2_1_8_1","doi-asserted-by":"crossref","first-page":"759","DOI":"10.1016\/0006-291X(74)91022-5","volume":"61","year":"1974","unstructured":"MATALON, R, MORQUIOS SYNDROME - DEFICIENCY OF A CHONDROITIN SULFATE N-ACETYLHEXOSAMINE SULFATE SULFATASE, BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS 61: 759 (1974).","journal-title":"BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS"},{"key":"e_1_2_1_9_1","unstructured":"McKusick V. A. Heritable Disorders of Connective Tissue 4: 583 (1972)."},{"key":"e_1_2_1_10_1","doi-asserted-by":"crossref","first-page":"847","DOI":"10.3181\/00379727-110-27668","volume":"110","year":"1962","unstructured":"PEDRINI, V, ISOLATION AND IDENTIFICATION OF KERATOSULPHATE IN URINE OF PATIENTS AFFECTED BY MORQUIO-ULLRICH DISEASE, PROCEEDINGS OF THE SOCIETY FOR EXPERIMENTAL BIOLOGY AND MEDICINE 110: 847 (1962).","journal-title":"PROCEEDINGS OF THE SOCIETY FOR EXPERIMENTAL BIOLOGY AND MEDICINE"},{"key":"e_1_2_1_11_1","first-page":"465","volume":"84","year":"1974","unstructured":"PEDRINIMILLE, A, GLYCOSAMINOGLYCANS OF ILIAC CREST CARTILAGE IN NORMAL CHILDREN AND IN MORQUIOS DISEASE, JOURNAL OF LABORATORY AND CLINICAL MEDICINE 84: 465 (1974).","journal-title":"JOURNAL OF LABORATORY AND CLINICAL MEDICINE"},{"key":"e_1_2_1_12_1","doi-asserted-by":"crossref","first-page":"1036","DOI":"10.1172\/JCI108345","volume":"57","year":"1976","unstructured":"SINGH, J, N-ACETYLGALACTOSAMINE-6-SULFATE SULFATASE IN MAN - ABSENCE OF ENZYME IN MORQUIO DISEASE, JOURNAL OF CLINICAL INVESTIGATION 57: 1036 (1976).","journal-title":"JOURNAL OF CLINICAL INVESTIGATION"},{"key":"e_1_2_1_13_1","first-page":"438","volume":"84","year":"1974","unstructured":"SINGH, J, SANFILIPPO DISEASE - DIFFERENTIATION OF TYPES A AND B BY AN ANALYTICAL METHOD, JOURNAL OF LABORATORY AND CLINICAL MEDICINE 84: 438 (1974).","journal-title":"JOURNAL OF LABORATORY AND CLINICAL MEDICINE"},{"key":"e_1_2_1_14_1","doi-asserted-by":"crossref","first-page":"267","DOI":"10.1016\/S0021-9258(18)69513-4","volume":"235","year":"1960","unstructured":"SUZUKI, S, ENZYMATIC SULFATION OF MUCOPOLYSACCHARIDES IN HEN OVIDUCT .2. MECHANISM OF THE REACTION STUDIED WITH OLIGOSACCHARIDES AND MONOSACCHARIDES AS ACCEPTORS, JOURNAL OF BIOLOGICAL CHEMISTRY 235: 267 (1960).","journal-title":"JOURNAL OF BIOLOGICAL CHEMISTRY"}],"container-title":["Science"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.science.org\/doi\/pdf\/10.1126\/science.199.4324.79","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,1,12]],"date-time":"2024-01-12T04:10:43Z","timestamp":1705032643000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.science.org\/doi\/10.1126\/science.199.4324.79"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1978,1,6]]},"references-count":14,"journal-issue":{"issue":"4324","published-print":{"date-parts":[[1978,1,6]]}},"alternative-id":["10.1126\/science.199.4324.79"],"URL":"https:\/\/doi.org\/10.1126\/science.199.4324.79","relation":{},"ISSN":["0036-8075","1095-9203"],"issn-type":[{"value":"0036-8075","type":"print"},{"value":"1095-9203","type":"electronic"}],"subject":[],"published":{"date-parts":[[1978,1,6]]}}}