Deficient phosphorylation of mannose residues of mannan in fibroblasts of patients with mucolipidoses II and III
- 1 February 1981
- journal article
- research article
- Published by Portland Press Ltd. in Biochemical Journal
- Vol. 193 (2) , 651-654
- https://doi.org/10.1042/bj1930651
Abstract
Incorporation of 32P from [gamma 32P]ATP into mannan could not be detected in homogenates of cultivated skin fibroblasts from patients with mucolipidosis II, and accounted for only up to 10% of normal control activity in cell lysates from patients with mucolipidosis III. Parents of patients with mucolipidosis II demonstrated 60-70% of normal control activity. On high-voltage electrophoresis, the hydrolysed mannan from reactions performed with normal cells, over the pH range 5.5-7.5, yielded a radioactive band migrating with the same mobility as mannose 6-phosphate, whereas no such product could be demonstrated in fibroblasts of patients with mucolipidosis II.This publication has 18 references indexed in Scilit:
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