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Inter cellular exchange of lysosomal enzymes enzyme assays in single human fibroblasts after co cultivation


, : Inter cellular exchange of lysosomal enzymes enzyme assays in single human fibroblasts after co cultivation. Biochemical and Biophysical Research Communications 69(2): 311-318

Intercellular exchange of N-acetyl-.beta.-D-glucosaminidase (EC 3.2.1.30), .beta.-galactosidase (EC 3.2.1.23) and acid .alpha.-glucosidase (EC 3.2.1.20) was studied after co-cultivation of normal and enzyme deficient human fibroblasts in confluent cultures. Enzyme activities were measured in single cells using microchemical procedures. After co-cultivation of normal control fibroblasts and those from a patient with Sandhoff's disease an increase of activity of N-acetyl-.beta.-D-glucosaminidase was found in Sandhoff cells, together with a decrease of activity in normal control cells. After co-cultivation of normal fibroblasts and those from patients with glycogenosis II and GM1-gangliosidosis, no indication was found for intercellular transfer of acid .alpha.-glucosidase and .beta.-galactosidase respectively. The significance of the results is discussed in respect of the hypothesis of Hickman and Neufeld about secretion and uptake of lysosomal enzymes.

US$29.90

PMID: 1063570


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