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Synthesis and characterization of a new fluorogenic substrate for alpha-galactosidase
Alpha-galactosidase A hydrolyzes the terminal alpha-galactosyl moieties from glycolipids and glycoproteins in lysosomes. Mutations in α-galactosidase cause lysosomal accumulation of the glycosphingolipid, globotriaosylceramide, which leads to Fabry disease. Small-molecule chaperones that bind to mut...
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| Main Authors: | , , , , , , , , |
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| Format: | Artigo |
| Sprog: | Inglês |
| Udgivet: |
2009
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| Fag: | |
| Online adgang: | https://ncbi.nlm.nih.gov/pmc/articles/PMC2756488/ https://ncbi.nlm.nih.gov/pubmed/19521690 https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1007/s00216-009-2879-5 |
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