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The Entrapment of Mannose-Terminated Glucocerebrosidase (Alglucerase) in Human Carrier Erythrocytes

  • Bridget E. Bax
  • Murray D. Bain
  • Chandra P. Ward
  • Anthony H. Fensom
  • Ronald A. Chalmers

Abstract

The enzyme glucocerebrosidase (β-D-glucosyl-N-acylsphingosine glucohydrolase, EC 3.2.1.45) 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.

Keywords

Enzyme Replacement Therapy Gauche Disease Lysosomal Storage Disorder Sodium Taurocholate Stock Concentration 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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References

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Copyright information

© Springer Science+Business Media New York 1997

Authors and Affiliations

  • Bridget E. Bax
    • 1
  • Murray D. Bain
    • 1
  • Chandra P. Ward
    • 2
  • Anthony H. Fensom
    • 2
  • Ronald A. Chalmers
    • 1
  1. 1.Paediatric Metabolism Unit, Department of Child HealthSt George’s Hospital Medical SchoolLondonUK
  2. 2.SAS Laboratory for Genetic Enzyme DefectsGuy’s HospitalLondonUK

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