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Glucocerebrosidase, a Membrane-Associated Lysosomal Enzyme Deficient in Gaucher Disease

  • A. W. Schram
  • J. M. F. G. Aerts
  • S. van Weely
  • J. A. Barranger
  • J. M. Tager
Part of the Methodological Surveys in Biochemistry and Analysis book series (MSBA, volume 17)

Abstract

Lysosomal storage diseases are characterized by a-massive intralysosomal accumulation of undegraded material, causing dysfunction of the cells involved [1]. The storage can have different causes, viz. (1)a generalized deficiency of one or more enzymes in the lysosomes, (2) deficiency of a cofactor required for the stability of one or more enzymes, or (3) deficiency of a cofactor required for expression of activity in vivo [2–5].

Keywords

Sialic Acid Lysosomal Enzyme Gauche Disease None None Artificial Substrate 
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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Copyright information

© Plenum Press, New York 1987

Authors and Affiliations

  • A. W. Schram
    • 1
  • J. M. F. G. Aerts
    • 1
  • S. van Weely
    • 1
  • J. A. Barranger
    • 2
  • J. M. Tager
    • 1
  1. 1.Laboratory of BiochemistryUniversity of AmsterdamAmsterdamThe Netherlands
  2. 2.Division of Human Genetics Children’s Hospital of Los AngelesUniversity of Southern CaliforniaLos AngelesUSA

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