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Science 25 November 1977:
Vol. 198. no. 4319, pp. 834 - 836
DOI: 10.1126/science.144321
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Articles

Science, Vol 198, Issue 4319, 834-836
Copyright © 1977 by American Association for the Advancement of Science

articles

Mucopolysaccharidosis in a cat with arylsulfatase B deficiency: a model of Maroteaux-Lamy syndrome

PF Jezyk, ME Haskins, DF Patterson, WJ Mellman, and M Greenstein

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.


THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES:
Mechanism of Glycosaminoglycan-Mediated Bone and Joint Disease: Implications for the Mucopolysaccharidoses and Other Connective Tissue Diseases.
C. M. Simonaro, M. D'Angelo, X. He, E. Eliyahu, N. Shtraizent, M. E. Haskins, and E. H. Schuchman (2008)
Am. J. Pathol. 172, 112-122
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Management Guidelines for Mucopolysaccharidosis VI.
R. Giugliani, P. Harmatz, and J. E. Wraith (2007)
Pediatrics 120, 405-418
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Mucopolysaccharidosis VII in a Cat.
P. C. Schultheiss, S. A. Gardner, J. M. Owens, D. A. Wenger, and M. A. Thrall (2000)
Vet. Pathol. 37, 502-505
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Advantages of Using Same Species Enzyme for Replacement Therapy in a Feline Model of Mucopolysaccharidosis Type VI.
J. Bielicki, A. C. Crawley, R. C. A. Davey, J. C. Varnai, and J. J. Hopwood (1999)
J. Biol. Chem. 274, 36335-36343
   Abstract »    Full Text »    PDF »
Niemann-Pick disease: a genetic model in Siamese cats.
D. Wenger, M Sattler, T Kudoh, S. Snyder, and R. Kingston (1980)
Science 208, 1471-1473
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