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Gaucher disease (type 1): Physical and kinetic properties of liposomal and soluble ‘acid’ β-glucosidase

  • Published:
Journal of Inherited Metabolic Disease

Abstract

‘Acid’ β-glucosidase of human spleen, from either normal controls or patients with type 1 (adult) Gaucher disease, was incorporated into phosphatidylcholine liposomes. The non-incorporated (soluble) Gaucherenzyme had a higher apparent molecular weight than had the corresponding control. Liposomal ‘acid’ β-glucosidase prepared from Gaucher-spleen was more thermostable than was the corresponding normal enzyme; it was also stimulated by acidic lipids to a much lesser extent. The results suggest that the genetic mutation in type 1 (adult) Gaucher disease has multiple effects on the glycoprotein form of ‘acid’ β-glucosidase.

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Authors and Affiliations

  1. Department of Biochemistry, London Hospital Medical College, E1 2AD, London, UK

    M. Carroll

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  1. M. Carroll
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Carroll, M. Gaucher disease (type 1): Physical and kinetic properties of liposomal and soluble ‘acid’ β-glucosidase. J Inherit Metab Dis 8, 33–37 (1985). https://doi.org/10.1007/BF01805482

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  • DOI: https://doi.org/10.1007/BF01805482

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