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sphingolipidoses

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sphingolipidoses
A group of inborn errors of sphingolipid metabolism in which lysosphingolipids accumulate, inhibiting protein kinase C activity in signal transduction, cell differentiation and in tumor promotion. See Ceramide lactoside lipidosis, Fabry's disease, Fucosidosis, Gaucher disease, Gangliosidosis, Globoid cell dystrophy, Krabbe's disease, Metachromatic leukodystrophy, Niemann-Pick disease, Sanhoff's disease, Tay-Sachs disease.


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