Elsevier

Clinica Chimica Acta

Volume 182, Issue 1, 15 June 1989, Pages 41-52
Clinica Chimica Acta

Research communication
Effects of N-hydroxyethyl-1-deoxynojirimycin (BAY m 1099) on the activity of neutral- and acid α-glucosidases in human fibroblasts and HepG2 cells

https://doi.org/10.1016/0009-8981(89)90148-4Get rights and content

Abstract

The effect of the glucose analogue N-hydroxyethyl-1-deoxynojirimycin (BAY m 1099) on the activity of α-glucosidases was studied in human fibroblasts and HepG2 cells. BAY m 1099 inhibits neutral and acid α-glucosidase activities of both cell types in a dosage-dependent and reversible manner. Inhibition of endoplasmic reticulum glucosidases I and/or II is suggested by delayed processing of lysosomal (acid) α-glucosidase. Competitive inhibition of mature acid α-glucosidase leads to lysosomal accumulation of glycogen as in glycogenosis type II. There seems to be little risk, however, of inducing this storage disorder when using the drug in a dose of 50 mg per os for treatment of type II diabetes. In high doses, the drug may prove useful for studying the pathogenesis of glycogenosis type II in vitro or in animal models.

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