The Entrapment of Mannose-Terminated Glucocerebrosidase (Alglucerase) in Human Carrier Erythrocytes
The enzyme glucocerebrosidase (β-D-glucosyl-N-acylsphingosine glucohydrolase, EC 22.214.171.124) is responsible for the hydrolytic cleavage of glucose from the glycolipid glucocerebroside within the lysosomes of tissue macrophages. Gaucher’s disease is an autosomal recessive, lysosomal storage disorder and is due to a deficiency of glucocerebrosidase. An absence of glucocereobrosidase results in an accumulation of glucocere-broside within the macrophages of the reticulo-endothelial system in the spleen, liver and bone marrow to produce Gaucher cells which are characteristic of this disorder.
KeywordsEnzyme Replacement Therapy Gauche Disease Lysosomal Storage Disorder Sodium Taurocholate Stock Concentration
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- Beutler, E. Gaucher’s disease. New Engl. J. Med. 25:1354–1360 (1991).Google Scholar
- Chalmers R.A. Comparison and potential of hypo-osmotic and iso-osmotic erythrocyte ghosts and carrier erythrocytes as drug and enzyme carriers. Biblthca. Haemat. 51: 15–24 (1985).Google Scholar
- Sprandel, U., Hubbard, A.R. and Chalmers, R.A. Survival of ‘carrier erythrocytes’ in dogs. Clin. Sci. 59: 7P (1980).Google Scholar