For research use only. Not for therapeutic Use.
Isofagomine (D-<wbr></wbr>tartrate) is a competitive inhibitor of human lysosomal β-<wbr></wbr>glucosidase (K<sub>i</sub> = 0.016-<wbr></wbr>0.025 µM; IC<sub>50</sub> = 0.06 µM). By interacting with the catalytic pocket of β-<wbr></wbr>glucosidase it acts as a chemical chaperone that increases the amount of β-<wbr></wbr>glucosidase by stabilizing and/or promoting the folding of the enzyme. Isofagomine (D-<wbr></wbr>tartrate) has been shown to increase lysosomal β-<wbr></wbr>glucosidase activity by 2-<wbr></wbr> to 3-<wbr></wbr>fold in mutant N370S Gaucher fibroblasts. This compound has been studied in the context of Gaucher disease, a lysosomal storage disorder resulting from substantial deficiency of β-<wbr></wbr>glucosidase and recently identified as a parkinsonism risk factor.
| Catalog Number | R009129 |
| CAS Number | 957230-65-8 |
| Synonyms | (3R,4R,5R)-5-(Hydroxymethyl)-3,4-piperidinediol ?(2S,3S)-2,3-Dihydroxybutanedioate |
| Molecular Formula | C6H13NO3 • C4H6O6 |
| Purity | ≥95% |
| Target | Glucosidase |
| Storage | -20°C |
| InChIKey | ULBPPCHRAVUQMC-RWOHWRPJSA-N |
