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

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Franken, S; Wittke, D; Mansson, JE; D'Hooge, R; De Deyn, PP; Lüllmann-Rauch, R; Matzner, U; Gieselmann, V;
  • Publisher: BioMed Central
  • Journal: Lipids in Health and Disease,volume 5,pages21-21 (issn: 1476-511X, eissn: 1476-511X)
  • Related identifiers: pmc: PMC1564137, doi: 10.1186/1476-511X-5-21
  • Subject: Nutritional diseases. Deficiency diseases | RC620-627 | Research

<p>Summary</p> <p>Background</p> <p>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 as... View more
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