Mucopolysaccharidosis: Secondarily Induced Abnormal Distribution of Lysosomal Isoenzymes

doi:10.1126/science.181.4097.352

Account Information

Guest Alerts | Access Rights | My Account | Sign In

Site Navigation

Article Views

Article Tools

My Science

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

Prev | Table of Contents | Next

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 patientswith mucopolysaccharidosis are decreased for $beta$ -galactosidase, $agr$ -galactosidase, and arylsulfatase A; total activities of fourother hydrolases are normal or increased. The isoenzyme distributionof five hydrolases ( $beta$ -glucuronidase, $agr$ -glucosidase, $beta$ - galactosidase,N-acetyl- $beta$ -glucosaminidase, and $agr$ -galactosidase) is ábnormalin that the isoelectric points (by isoelectric focusing) ofthese enzymes are more acid than in control liver. Along withthe isoenzyme abnormalities different kinds of glycolipids werestored in kidney, liver, and brain. The isoenzyme abnormalitiescan be reproduced in vitro by addition of chondroitin sulfateto a homogenate of normal liver, suggesting that stable bindingoccurs between mucopolysaccharides and the hydrolase molecules.After the addition of chondroitin sulfate, the total activityof $beta$ -galactosidase is inhibited, whereas other hydrolases areaffected only slightly or not at all.

THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES:

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
| Abstract » | PDF »