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Preliminary Characterization of a Ganglioside Sialidase in Normal and Mucolipidosis IV Fibroblasts

  • M. Lieser
  • E. Harms
  • H. Kern
  • M. Cantz
Part of the NATO ASI Series book series (NSSA, volume 116)

Abstract

A ganglioside sialidase deficiency detected in cultured skin fibroblasts of patients with mucolipidosis IV (ML IV) has been suggested to be the primary cause of this autosomal recessive disorder, and a lysosomal storage of gangliosides GM3, GD3, phospholipids and mucopolysaccharides has been shown to occur1–5. Up to now ganglioside-specific sialidase activity has been found in the plasma membrane but not in lysosomes, where ganglioside catabolism is thought to take place. Therefore, we reinvestigated the subcellular distribution of ganglioside sialidase using free-flow electrophoresis.

Keywords

Culture Skin Fibroblast Sialidase Activity Plasma Membrane Marker Skin Fibroblast Derive Fibroblast Homogenate 
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

© Plenum Press, New York 1986

Authors and Affiliations

  • M. Lieser
    • 1
  • E. Harms
    • 2
  • H. Kern
    • 2
  • M. Cantz
    • 1
  1. 1.Insitut für Pathochemie und allgemeine Neurochemie der UniversitätHeidelbergDeutschland
  2. 2.Kinderklinik der TechnischenUniversität MünchenMünchen 40Deutschland

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