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Science 27 July 1973:
Vol. 181. no. 4097, pp. 352 - 354
DOI: 10.1126/science.181.4097.352
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Articles

Mucopolysaccharidosis: Secondarily Induced Abnormal Distribution of Lysosomal Isoenzymes

J. A. Kint 1, G. Dacremont 1, D. Carton 1, E. Orye 1, and C. Hooft 1

1 Department of Pediatrics, Akademisch Ziekenhuis, Rijksuniversiteit, B-9000 Ghent, Belgium

Total activities of acid hydrolases in liver of two patients with mucopolysaccharidosis are decreased for beta-galactosidase, agr-galactosidase, and arylsulfatase A; total activities of four other hydrolases are normal or increased. The isoenzyme distribution of five hydrolases (beta-glucuronidase, agr-glucosidase, beta- galactosidase, N-acetyl-beta-glucosaminidase, and agr-galactosidase) is ábnormal 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 beta-galactosidase is inhibited, whereas other hydrolases are affected only slightly or not at all.


THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES:
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M. A. Lavery, W. R. Green, E. W. Jabs, M. W. Luckenbach, and J. L. Cox (1983)
Arch Ophthalmol 101, 1263-1274
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Pathologic Findings in Mucopolysaccharidosis Type IIIB (Sanfilippo's Syndrome B).
M. G. Hadfield, N. R. Ghatak, I. Nakoneczna, H. R. Lippman, E. C. Myer, G. Constantopoulos, and R. M. Bradley (1980)
Arch Neurol 37, 645-650
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Lactosyl ceramidosis: normal activity for two lactosyl ceramide beta-galactosidases.
D. Wenger, M Sattler, C Clark, H Tanaka, K Suzuki, and G Dawson (1975)
Science 188, 1310-1312
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