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The effects of pH and iminosugar pharmacological chaperones on lysosomal glycosidase structure and stability
Human lysosomal enzymes acid-β-glucosidase (GCase) and acid-α-galactosidase (α-Gal A) hydrolyze, respectively, the sphingolipids glucosyl- and globotriaosyl- ceramide, and mutations in these enzymes lead to the lipid metabolism disorders Gaucher and Fabry disease. We have investigated the structure...
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| Autors principals: | , , , |
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| Format: | Artigo |
| Idioma: | Inglês |
| Publicat: |
2009
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| Matèries: | |
| Accés en línia: | https://ncbi.nlm.nih.gov/pmc/articles/PMC2699628/ https://ncbi.nlm.nih.gov/pubmed/19374450 https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1021/bi9002265 |
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