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Modest phenotypic improvements in ASA-deficient mice with only one UDP-galactose:ceramide-galactosyltransferase gene

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 mated...

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Detalles Bibliográficos
Autores principales:	Franken, S, Wittke, D, Mansson, JE, D'Hooge, R, De Deyn, PP, Lüllmann-Rauch, R, Matzner, U, Gieselmann, V
Formato:	Texto
Lenguaje:	English
Publicado:	BioMed Central 2006
Materias:	Research
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1564137/ https://www.ncbi.nlm.nih.gov/pubmed/16893448 http://dx.doi.org/10.1186/1476-511X-5-21

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