{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T09:52:38Z","timestamp":1790070758989,"version":"4.0.1"},"reference-count":30,"publisher":"American Association for the Advancement of Science (AAAS)","issue":"4319","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Science"],"published-print":{"date-parts":[[1977,11,25]]},"abstract":"<jats:p>A Siamese cat that presented clinical signs similar to those seen in humans with mucopolysaccharidoses was studied. The animal excreted increased amounts of polymeric glycosaminoglycans in the urine, consisting almost entirely of dermatan sulfate. Electron microscopy of circulating polymorphonuclear leukocytes revealed the presence of many membrane-bound lamellar inclusion bodies. Sulfate incorporation studies with cultured skin fibroblasts indicated defective glycosaminoglycan degradation. These cells showed a deficiency in arylsulfatase B activity. The disorder appears similar or identical to the Maroteaux-Lamy syndrome described in humans.<\/jats:p>","DOI":"10.1126\/science.144321","type":"journal-article","created":{"date-parts":[[2006,10,5]],"date-time":"2006-10-05T16:03:07Z","timestamp":1160064187000},"page":"834-836","source":"Crossref","is-referenced-by-count":95,"title":["Mucopolysaccharidosis in a Cat with Arylsulfatase B Deficiency: A Model of Maroteaux-Lamy Syndrome"],"prefix":"10.1126","volume":"198","author":[{"given":"Peter F.","family":"Jezyk","sequence":"first","affiliation":[{"name":"Section of Medical Genetics, University of Pennsylvania School of Veterinary Medicine, and the University of Pennsylvania Genetics Center, Philadelphia 19174"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mark E.","family":"Haskins","sequence":"additional","affiliation":[{"name":"Section of Medical Genetics, University of Pennsylvania School of Veterinary Medicine, and the University of Pennsylvania Genetics Center, Philadelphia 19174"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Donald F.","family":"Patterson","sequence":"additional","affiliation":[{"name":"Section of Medical Genetics, University of Pennsylvania School of Veterinary Medicine, and the University of Pennsylvania Genetics Center, Philadelphia 19174"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"William J.","family":"Mellman","sequence":"additional","affiliation":[{"name":"Department of Human Genetics, University of Pennsylvania School of Medicine, Philadelphia 19174"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Greenstein","sequence":"additional","affiliation":[{"name":"Department of Human Genetics, University of Pennsylvania School of Medicine, Philadelphia 19174"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"221","reference":[{"key":"e_1_2_1_1_1","first-page":"1","volume":"93","year":"1972","unstructured":"BELCHER, R.W., ULTRASTRUCTURE AND CYTOCHEMISTRY OF LYMPHOCYTES IN GENETIC MUCOPOLYSACCHARIDOSES, ARCHIVES OF PATHOLOGY 93: 1 (1972).","journal-title":"ARCHIVES OF PATHOLOGY"},{"key":"e_1_2_1_2_1","doi-asserted-by":"crossref","first-page":"475","DOI":"10.1203\/00006450-197505000-00003","volume":"9","year":"1975","unstructured":"BERATIS, N.G., ARYLSULFATASE B DEFICIENCY IN MAROTEAUX-LAMY SYNDROME - CELLULAR STUDIES AND CARRIER IDENTIFICATION, PEDIATRIC RESEARCH 9: 475 (1975).","journal-title":"PEDIATRIC RESEARCH"},{"key":"e_1_2_1_3_1","doi-asserted-by":"crossref","first-page":"541","DOI":"10.1016\/S0021-9258(18)64700-3","volume":"233","year":"1958","unstructured":"CIFONELLI, J.A., CHEMISTRY OF BETA-HEPARIN (CHONDROITINSULFURIC ACID-B), JOURNAL OF BIOLOGICAL CHEMISTRY 233: 541 (1958).","journal-title":"JOURNAL OF BIOLOGICAL CHEMISTRY"},{"key":"e_1_2_1_4_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.404709"},{"key":"e_1_2_1_5_1","first-page":"334","volume":"169","year":"1976","unstructured":"COWELL, K.R., MUCOPOLYSACCHARIDOSIS IN A CAT, JOURNAL OF THE AMERICAN VETERINARY MEDICAL ASSOCIATION 169: 334 (1976).","journal-title":"JOURNAL OF THE AMERICAN VETERINARY MEDICAL ASSOCIATION"},{"key":"e_1_2_1_6_1","doi-asserted-by":"crossref","first-page":"455","DOI":"10.1016\/S0006-291X(74)80001-X","volume":"59","year":"1974","unstructured":"FLUHARTY, A.L., ARYLSULFATASE-B DEFICIENCY IN MAROTEAUX-LAMY SYNDROME CULTURED FIBROBLASTS, BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS 59: 455 (1974).","journal-title":"BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS"},{"key":"e_1_2_1_7_1","first-page":"699","volume":"60","year":"1968","unstructured":"FRATANTONI, J.C., DEFECT IN HURLERS AND HUNTERS SYNDROMES - FAULTY DEGRADATION OF MUCOPOLYSACCHARIDE, PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA 60: 699 (1968).","journal-title":"PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA"},{"key":"e_1_2_1_8_1","first-page":"969","volume":"82","year":"1973","unstructured":"GLASER, J.H., BETA-GLUCURONIDASE DEFICIENCY MUCOPOLYSACCHARIDOSIS - METHODS FOR ENZYMATIC DIAGNOSIS, JOURNAL OF LABORATORY AND CLINICAL MEDICINE 82: 969 (1973).","journal-title":"JOURNAL OF LABORATORY AND CLINICAL MEDICINE"},{"key":"e_1_2_1_9_1","first-page":"598","volume":"33","year":"1974","unstructured":"HAMILTON, M.J., FED P FED AM SOC EXP 33: 598 (1974).","journal-title":"FED P FED AM SOC EXP"},{"key":"e_1_2_1_10_1","doi-asserted-by":"crossref","first-page":"141","DOI":"10.1016\/0014-4800(69)90036-7","volume":"10","year":"1969","unstructured":"HAUST, M.D., FINE STRUCTURE OF LIVER IN CHILDREN WITH HURLERS SYNDROME, EXPERIMENTAL AND MOLECULAR PATHOLOGY 10: 141 (1969).","journal-title":"EXPERIMENTAL AND MOLECULAR PATHOLOGY"},{"key":"e_1_2_1_11_1","first-page":"821","volume":"32","year":"1973","unstructured":"HEGREBERG, G.A., INHERITED STORAGE-DISEASE OF CATS, FEDERATION PROCEEDINGS 32: 821 (1973).","journal-title":"FEDERATION PROCEEDINGS"},{"key":"e_1_2_1_12_1","doi-asserted-by":"crossref","first-page":"391","DOI":"10.1136\/vr.92.15.391","volume":"92","year":"1973","unstructured":"JOLLY, R.D., INHERITED LYSOSOMAL STORAGE DISEASES - ESSAY IN COMPARATIVE MEDICINE, VETERINARY RECORD 92: 391 (1973).","journal-title":"VETERINARY RECORD"},{"key":"e_1_2_1_13_1","doi-asserted-by":"crossref","first-page":"1336","DOI":"10.1056\/NEJM197006112822403","volume":"282","year":"1970","unstructured":"KABACK, M.M., NEW ENGLAND JOURNAL OF MEDICINE 282: 1336 (1970).","journal-title":"NEW ENGLAND JOURNAL OF MEDICINE"},{"key":"e_1_2_1_14_1","first-page":"309","volume":"77","year":"1975","unstructured":"KINOSHITA, T, JOURNAL OF BIOCHEMISTRY 77: 309 (1975).","journal-title":"JOURNAL OF BIOCHEMISTRY"},{"key":"e_1_2_1_15_1","doi-asserted-by":"crossref","first-page":"256","DOI":"10.1016\/0006-2944(74)90060-X","volume":"9","year":"1974","unstructured":"LAKATOS, M, SIMPLE MEASUREMENT OF URINARY GLYCOSAMINOGLYCANS AND DEGRADATION PRODUCTS, BIOCHEMICAL MEDICINE 9: 256 (1974).","journal-title":"BIOCHEMICAL MEDICINE"},{"key":"e_1_2_1_16_1","doi-asserted-by":"crossref","first-page":"860","DOI":"10.1016\/S0022-3476(68)80239-2","volume":"73","year":"1968","unstructured":"LOEB, H, BIOCHEMICAL AND ULTRASTRUCTURAL STUDIES IN HURLERS SYNDROME, JOURNAL OF PEDIATRICS 73: 860 (1968).","journal-title":"JOURNAL OF PEDIATRICS"},{"key":"e_1_2_1_17_1","doi-asserted-by":"crossref","first-page":"312","DOI":"10.1016\/S0022-3476(65)80256-6","volume":"67","year":"1965","unstructured":"MAROTEAUX, P, HURLERS DISEASE MORQUIOS DISEASE AND RELATED MUCOPOLYSACCHARIDOSES, JOURNAL OF PEDIATRICS 67: 312 (1965).","journal-title":"JOURNAL OF PEDIATRICS"},{"key":"e_1_2_1_18_1","first-page":"1849","volume":"71","year":"1963","unstructured":"MAROTEAUX, P, UNE NOUVELLE DYSOSTOSE AVEC ELIMINATION URINAIRE DE CHONDROITINE-SULFATE B, PRESSE MEDICALE 71: 1849 (1963).","journal-title":"PRESSE MEDICALE"},{"key":"e_1_2_1_19_1","first-page":"611","year":"1972","unstructured":"MCKUSICK, V.A., HERITABLE DISORDERS: 611 (1972).","journal-title":"HERITABLE DISORDERS"},{"key":"e_1_2_1_20_1","unstructured":"NEUFELD E.F. COMMUNICATION."},{"key":"e_1_2_1_21_1","doi-asserted-by":"crossref","first-page":"169","DOI":"10.1136\/jmg.13.3.169","volume":"13","year":"1976","unstructured":"PENNOCK, C.A., MUCOPOLYSACCHARIDOSES, JOURNAL OF MEDICAL GENETICS 13: 169 (1976).","journal-title":"JOURNAL OF MEDICAL GENETICS"},{"key":"e_1_2_1_22_1","doi-asserted-by":"publisher","DOI":"10.1126\/science.824730"},{"key":"e_1_2_1_23_1","doi-asserted-by":"crossref","first-page":"1536","DOI":"10.1016\/S0021-9258(18)93575-1","volume":"243","year":"1968","unstructured":"SAITO, H, ENZYMATIC METHODS FOR DETERMINATION OF SMALL QUANTITIES OF ISOMERIC CHONDROITIN SULFATES, JOURNAL OF BIOLOGICAL CHEMISTRY 243: 1536 (1968).","journal-title":"JOURNAL OF BIOLOGICAL CHEMISTRY"},{"key":"e_1_2_1_24_1","doi-asserted-by":"crossref","first-page":"73","DOI":"10.1007\/BF00685324","volume":"30","year":"1974","unstructured":"SHEHAN, B.J., ACTA NEUROPATHOL 30: 73 (1974).","journal-title":"ACTA NEUROPATHOL"},{"key":"e_1_2_1_25_1","first-page":"337","volume":"25","year":"1970","unstructured":"SPRANGER, J.W., MUCOPOLYSACCHARIDOSIS VI (MAROTEAUX-LAMYS DISEASE), HELVETICA PAEDIATRICA ACTA 25: 337 (1970).","journal-title":"HELVETICA PAEDIATRICA ACTA"},{"key":"e_1_2_1_26_1","doi-asserted-by":"crossref","first-page":"747","DOI":"10.1001\/archpedi.1973.02110190597003","volume":"126","year":"1973","unstructured":"STUMPF, D.A., MUCOPOLYSACCHARIDOSIS TYPE-VI - (MAROTEAUX-LAMY SYNDROME) .1. SULFATASE-B DEFICIENCY IN TISSUES, AMERICAN JOURNAL OF DISEASES OF CHILDREN 126: 747 (1973).","journal-title":"AMERICAN JOURNAL OF DISEASES OF CHILDREN"},{"key":"e_1_2_1_27_1","doi-asserted-by":"crossref","first-page":"241","DOI":"10.1016\/0006-2944(75)90042-3","volume":"14","year":"1975","unstructured":"TANIGUCHI, N, DIAGNOSIS OF GENETIC MUCOPOLYSACCHARIDOSES - ELECTROPHORETIC AND ENZYMATIC CHARACTERIZATION OF URINARY GLYCOSAMINOGLYCANS, BIOCHEMICAL MEDICINE 14: 241 (1975).","journal-title":"BIOCHEMICAL MEDICINE"},{"key":"e_1_2_1_28_1","first-page":"1142","volume":"19","year":"1966","unstructured":"TELLER, W, KLINISCHE WOCHENSCHRIFT 19: 1142 (1966).","journal-title":"KLINISCHE WOCHENSCHRIFT"},{"key":"e_1_2_1_29_1","first-page":"821","volume":"32","year":"1973","unstructured":"TOMCHICK, T.L., FAMILIAL LAFORAS DISEASE IN BEAGLE DOG, FEDERATION PROCEEDINGS 32: 821 (1973).","journal-title":"FEDERATION PROCEEDINGS"},{"key":"e_1_2_1_30_1","doi-asserted-by":"crossref","first-page":"439","DOI":"10.1016\/0003-2697(68)90205-4","volume":"26","year":"1968","unstructured":"WESSLER, E, ANALYTICAL AND PREPARATIVE SEPARATION OF ACIDIC GLYCOSAMINOGLYCANS BY ELECTROPHORESIS IN BARIUM ACETATE, ANALYTICAL BIOCHEMISTRY 26: 439 (1968).","journal-title":"ANALYTICAL BIOCHEMISTRY"}],"container-title":["Science"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.science.org\/doi\/pdf\/10.1126\/science.144321","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,1,11]],"date-time":"2024-01-11T03:28:57Z","timestamp":1704943737000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.science.org\/doi\/10.1126\/science.144321"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1977,11,25]]},"references-count":30,"journal-issue":{"issue":"4319","published-print":{"date-parts":[[1977,11,25]]}},"alternative-id":["10.1126\/science.144321"],"URL":"https:\/\/doi.org\/10.1126\/science.144321","relation":{},"ISSN":["0036-8075","1095-9203"],"issn-type":[{"value":"0036-8075","type":"print"},{"value":"1095-9203","type":"electronic"}],"subject":[],"published":{"date-parts":[[1977,11,25]]}}}