| Identification | Back Directory | [Name]
Isofagomine D-Tartrate | [CAS]
957230-65-8 | [Synonyms]
Isofagomine D-Tartrate
(3R,4R,5R)-5-(Hydroxymethyl)-3,4-piperidinediol (2S,3S)-2,3-Dihydroxybutanedioate | [Molecular Formula]
C10H19NO9 | [MOL File]
957230-65-8.mol | [Molecular Weight]
297.259 |
| Chemical Properties | Back Directory | [Appearance]
Off-White Solid | [Melting point ]
124-126°C | [storage temp. ]
-20°C Freezer, Under Inert Atmosphere | [solubility ]
Soluble in DMSO (up to 2 mg/ml) | [form ]
solid | [color ]
Off-white | [Stability:]
Stable for 1 year from date of purchase as supplied. Solutions in DMSO may be stored at -20°C for up to 1 month. |
| Hazard Information | Back Directory | [Chemical Properties]
Off-White Solid | [Uses]
Used as human acid ?glucosidase inhibitor and for the treatment of Gaucher disease | [Uses]
Used as human acid β-glucosidase inhibitor and for the treatment of Gaucher disease | [Description]
Isofagomine tartrate (957230-65-8) is a competitive inhibitor of human lysosomal β-glucosidase, IC50?= 0.06 μM.1?Increases the activity of N370S mutant acid β-glucosidase (GlcCerase) in Gaucher fibroblasts.2?Displays a direct pharmacological chaperone effect greatly increasing the amount of newly synthesized GlcCerase that traffics out of the ER.2 | [in vivo]
Vivo: Afegostat (AT2101) increases GCase activity in brain, liver, and spleen. Afegostat (100 mg/kg) administrates orally for 4 months to Thy1-aSyn mice improved motor and nonmotor function, abolishes microglial inflammatory response in the substantia nigra, reduces α-synuclein immunoreactivity in nigral dopaminergic neurons, and reduces the number of small α-synuclein aggregates, while increasing the number of large α-synuclein aggregates[1]. | [References]
1) Kuriyama?et al.?(2008),?In vitro inhibition of glycogen-degrading enzymes and glycosidases by six-membered sugar mimics and their evaluation in cell culture; Bioorg. Med. Chem.,?16?7330
2) Steet?et al. (2006),?The iminosugar isofagomine increases the activity of N370S mutant acid beta-glucosidase in Gaucher fibroblasts by several mechanisms; Proc. Natl. Acad. Sci. USA,?103?13813 |
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| Company Name: |
BOC Sciences
|
| Tel: |
16314854226 |
| Website: |
www.bocsci.com |
| Company Name: |
InvivoChem
|
| Tel: |
13549236410 |
| Website: |
https://www.invivochem.cn/ |
|