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Comparison of the alpha-mannosidases in fibroblast cultures from patients with mannosidosis and mucolipidosis II and from controls.

Authors
  • D J Halley
  • B G Winchester
  • L J Burditt
  • A d'Azzo
  • D Robinson
  • H Galjaard
Publication Date
May 01, 1980
Source
PMC
Keywords
Disciplines
  • Biology
License
Unknown

Abstract

The intracellular and extracellular acidic alpha-mannosidase in cultures of fibroblasts from mucolipidosis-II patients has normal kinetics. The extracellular activity in cultures of cells from mannosidosis patients is normal, but a mutant enzyme is associated with the cell surface and intracellular fraction. The results support the involvement of membrane cycling and recognition markers in lysosomal enzyme localization.

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