| Bioactivity | Afegostat hydrochloride (D-Isofagomine hydrochloride) is a potent β-galactosidase inhibitor with activity ameliorating GM1-gangliosidosis and Morquio B disease-associated mutations. Afegostat hydrochloride is able to induce the maturation of mutant β-galactosidase in fibroblasts from patients with GM1-gangliosidosis. Afegostat hydrochloride also promotes the reduction of keratin sulfate and oligosaccharide load in patient cells[1]. |
| CAS | 161302-93-8 |
| Formula | C6H14ClNO3 |
| Molar Mass | 183.63 |
| Transport | Room temperature in continental US; may vary elsewhere. |
| Storage | Please store the product under the recommended conditions in the Certificate of Analysis. |
| Reference | [1]. Richter F, et al. A GCase chaperone improves motor function in a mouse model of synucleinopathy. Neurotherapeutics. 2014 Oct;11(4):840-56. [2]. 4-epi-Isofagomine derivatives as pharmacological chaperones for the treatment of lysosomal diseases linked to β-galactosidase mutations: Improved synthesis and biological investigations |
Afegostat hydrochloride
CAS: 161302-93-8 F: C6H14ClNO3 W: 183.63
Afegostat hydrochloride (D-Isofagomine hydrochloride) is a potent β-galactosidase inhibitor with activity ameliorating
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