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A symptomatic Fabry disease mouse model generated by inducing globotriaosylceramide synthesis
Fabry disease is a lysosomal storage disorder in which neutral glycosphingolipids, predominantly globotriaosylceramide (Gb3), accumulate due to deficient α-galactosidase A (α-Gal A) activity. The α-Gal A-knockout (GLAko) mouse has been used as a model for Fabry disease, but it does not have any symp...
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| Hauptverfasser: | , , , , , , , |
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| Format: | Artigo |
| Sprache: | Inglês |
| Veröffentlicht: |
2013
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| Schlagworte: | |
| Online Zugang: | https://ncbi.nlm.nih.gov/pmc/articles/PMC4160053/ https://ncbi.nlm.nih.gov/pubmed/24094090 https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1042/BJ20130825 |
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