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GM2-Ganglioside Metabolism In Situ in Mucolipidosis IV Fibroblasts

โœ Scribed by S. Raghavan; E. Leshinsky; E.H. Kolodny

Book ID
110421226
Publisher
Springer
Year
1999
Tongue
English
Weight
911 KB
Volume
24
Category
Article
ISSN
0364-3190

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โœ G. M. S. Mancini; A. T. Hoogeveen; H. Galjaard; J. E. Mansson; L. Svennerholm ๐Ÿ“‚ Article ๐Ÿ“… 1986 ๐Ÿ› Springer ๐ŸŒ English โš– 585 KB

The uptake and catabolism of [3H-ceramide]-GM1 was followed in living fibroblasts from patient with different forms of beta-galactosidase deficiency. Gangliosides are identified according to the nomenclature of Svennerholm (1963). A total inability to metabolize the ingested substrate was found in i