Sulfatide Storage in Neurons Causes Hyperexcitability and Axonal Degeneration in a Mouse Model of Metachromatic Leukodystrophy

Metachromatic leukodystrophy is a lysosomal storage disorder caused by deficiency in the sulfolipid degrading enzyme arylsulfatase A (ASA). In the absence of a functional ASA gene, 3-O-sulfogalactosylceramide (sulfatide; SGalCer) and other sulfolipids accumulate. The storage is associated with progr...

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Detalhes bibliográficos
Publicado no:J Neurosci
Main Authors: Eckhardt, Matthias, Hedayati, Kerstin Khalaj, Pitsch, Julika, Lüllmann-Rauch, Renate, Beck, Heinz, Fewou, Simon Ngamli, Gieselmann, Volkmar
Formato: Artigo
Idioma:Inglês
Publicado em: Society for Neuroscience 2007
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Articles
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC6672212/
https://ncbi.nlm.nih.gov/pubmed/17715338
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1523/JNEUROSCI.2329-07.2007
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