Article
Modest phenotypic improvements in ASA-deficient mice with only one UDP-galactose:ceramide-galactosyltransferase gene.
Lipids in health and disease - 7 Aug 2006
Franken S, Wittke D, Mansson J E, D'Hooge R, De Deyn P P, Lüllmann-Rauch R, Matzner U, Gieselmann V
Abstract excerpt
BACKGROUND: Arylsulfatase A (ASA)-deficient mice are a model for the lysosomal storage disorder metachromatic leukodystrophy. This lipidosis is characterised by the lysosomal accumulation of the sphingolipid sulfatide. Storage of this lipid is associated with progressive demyelination. We have ma...
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