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...

Disgrifiad llawn

Wedi'i Gadw mewn:
Manylion Llyfryddiaeth
Prif Awduron: Franken, S, Wittke, D, Mansson, JE, D'Hooge, R, De Deyn, PP, Lüllmann-Rauch, R, Matzner, U, Gieselmann, V
Fformat: Artigo
Iaith:Inglês
Cyhoeddwyd: BioMed Central 2006
Pynciau:
Research
Mynediad Ar-lein:https://ncbi.nlm.nih.gov/pmc/articles/PMC1564137/
https://ncbi.nlm.nih.gov/pubmed/16893448
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1186/1476-511X-5-21
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