{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,27]],"date-time":"2026-09-27T01:36:13Z","timestamp":1790472973381,"version":"4.1.0"},"reference-count":20,"publisher":"American Association for the Advancement of Science (AAAS)","issue":"4097","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Science"],"published-print":{"date-parts":[[1973,7,27]]},"abstract":"<jats:p>\n            Total activities of acid hydrolases in liver of two patients with mucopolysaccharidosis are decreased for \u03b2-galactosidase, \u03b1-galactosidase, and arylsulfatase A; total activities of four other hydrolases are normal or increased. The isoenzyme distribution of five hydrolases (\u03b2-glucuronidase, \u03b1-glucosidase, \u03b2-galactosidase,\n            <jats:italic>N<\/jats:italic>\n            -acetyl-\u03b2-glucosaminidase, and \u03b1-galactosidase) is abnormal in that the isoelectric points (by isoelectric focusing) of these enzymes are more acid than in control liver. Along with the isoenzyme abnormalities different kinds of glycolipids were stored in kidney, liver, and brain. The isoenzyme abnormalities can be reproduced in vitro by addition of chondroitin sulfate to a homogenate of normal liver, suggesting that stable binding occurs between mucopolysaccharides and the hydrolase molecules. After the addition of chondroitin sulfate, the total activity of \u03b2-galactosidase is inhibited, whereas other hydrolases are affected only slightly or not at all.\n          <\/jats:p>","DOI":"10.1126\/science.181.4097.352","type":"journal-article","created":{"date-parts":[[2006,10,5]],"date-time":"2006-10-05T14:05:45Z","timestamp":1160057145000},"page":"352-354","source":"Crossref","is-referenced-by-count":79,"title":["Mucopolysaccharidosis: Secondarily Induced Abnormal Distribution of Lysosomal Isoenzymes"],"prefix":"10.1126","volume":"181","author":[{"given":"J. A.","family":"Kint","sequence":"first","affiliation":[{"name":"Department of Pediatrics, Akademisch Ziekenhuis, Rijksuniversiteit, B-9000 Ghent, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G.","family":"Dacremont","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Akademisch Ziekenhuis, Rijksuniversiteit, B-9000 Ghent, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D.","family":"Carton","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Akademisch Ziekenhuis, Rijksuniversiteit, B-9000 Ghent, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"E.","family":"Orye","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Akademisch Ziekenhuis, Rijksuniversiteit, B-9000 Ghent, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C.","family":"Hooft","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Akademisch Ziekenhuis, Rijksuniversiteit, B-9000 Ghent, Belgium"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"221","reference":[{"key":"e_1_2_1_1_1","doi-asserted-by":"crossref","first-page":"453","DOI":"10.1016\/0009-8981(59)90119-6","volume":"4","year":"1959","unstructured":"BAUM, H, THE ASSAY OF ARYLSULPHATASE-A AND ARYLSULPHATASE-B IN HUMAN URINE, CLINICA CHIMICA ACTA 4: 453 (1959).","journal-title":"CLINICA CHIMICA ACTA"},{"key":"e_1_2_1_2_1","doi-asserted-by":"crossref","first-page":"98","DOI":"10.1016\/0003-2697(67)90087-5","volume":"21","year":"1967","unstructured":"DIFERRANTE, N.M., MEASUREMENT OF URINARY MUCOPOLYSACCHARIDES, ANALYTICAL BIOCHEMISTRY 21: 98 (1967).","journal-title":"ANALYTICAL BIOCHEMISTRY"},{"key":"e_1_2_1_3_1","doi-asserted-by":"crossref","first-page":"324","DOI":"10.1007\/BF00440191","volume":"110","year":"1971","unstructured":"FURUSHO, K, ABNORMAL ARYLSULPHATASE ACTIVITIES OF FIBROBLASTS CULTURED FROM PATIENTS WITH MUCOPOLYSACCHARIDOSIS AND CYSTINOSIS, ZEITSCHRIFT FUR KINDERHEILKUNDE 110: 324 (1971).","journal-title":"ZEITSCHRIFT FUR KINDERHEILKUNDE"},{"key":"e_1_2_1_4_1","doi-asserted-by":"crossref","first-page":"68","DOI":"10.1016\/0014-5793(71)80667-1","volume":"13","year":"1971","unstructured":"GOLDSTONE, A, LYSOSOMAL HYDROLASES - CONVERSION OF ACIDIC TO BASIC FORMS BY NEURAMINIDASE, FEBS LETTERS 13: 68 (1971).","journal-title":"FEBS LETTERS"},{"key":"e_1_2_1_5_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.165.3893.611"},{"key":"e_1_2_1_6_1","first-page":"216","volume":"49","year":"1970","unstructured":"HULTBERG, B, SCAND J CLIN LAB INV 49: 216 (1970).","journal-title":"SCAND J CLIN LAB INV"},{"key":"e_1_2_1_7_1","first-page":"633","volume":"79","year":"1971","unstructured":"KINT, J, EXISTENCE AND ENZYMIC INTERCONVERSION OF ISOZYMES OF ALPHA-GALACTOSIDASE IN HUMAN ORGANS, ARCHIVES INTERNATIONALES DE PHYSIOLOGIE ET DE BIOCHIMIE 79: 633 (1971).","journal-title":"ARCHIVES INTERNATIONALES DE PHYSIOLOGIE ET DE BIOCHIMIE"},{"key":"e_1_2_1_8_1","unstructured":"KINT J.A. 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