Preliminary Characterization of a Ganglioside Sialidase in Normal and Mucolipidosis IV Fibroblasts
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.
KeywordsCulture Skin Fibroblast Sialidase Activity Plasma Membrane Marker Skin Fibroblast Derive Fibroblast Homogenate
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- 8.M. Zeigler and G. Bach, Ganglioside Sialidase Distribution in Mucolipidosis Type IV Cultured Fibroblasts, Arch. Biochem. Biophys. 241, (1985), in press.Google Scholar