| 产品详情 |
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| Product Name | 1-Deoxygalactonojirimycin (hydrochloride) |
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| Description | Purity >98%. Fabry disease is an X-linked hereditary lysosomal storage disorder caused by mutations in the Alpha-galactosidase gene that commonly lead to enzyme instability, misfolding, and degradation. 1-Deoxygalactonojirimycin is a competititve inhibitor of Alpha-galactosidase (IC50 = 40 nM). At subinhibitory concentrations, 1-Deoxygalactonojirimycin binds to Alpha-galactosidase and chaperones unstable enzyme variants through the endoplasmic reticulum, allowing its movement into lysosomes. Pharmacological chaperones, including 1-Deoxygalactonojirimycin, are used to promote lysosomal delivery of unstable proteins in lysosomal storage disorders, like Fabry disease. |
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| Size | n/a |
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| Concentration | n/a |
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| Applications | n/a |
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| Other Names | (2R,3S,4R,5S)-2-(hydroxymethyl)-3,4,5-piperidinetriol, monohydrochloride, DGJ, Migalastat |
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| Gene, Accession, CAS # | CAS: 75172-81-5 |
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| Catalog # | LS-H5883 |
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| Price | |
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| Order / More Info | 1-Deoxygalactonojirimycin (hydrochloride) from LIFESPAN BIOSCIENCES INC. |
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| Product Specific References | n/a |
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