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Isofagomine D-Tartrate

Names

[ CAS No. ]:
957230-65-8

[ Name ]:
Isofagomine D-Tartrate

[Synonym ]:
Isofagomine D-Tartrate
Butanedioic acid, 2,3-dihydroxy-, (2S,3S)-, compd. with (3R,4R,5R)-5-(hydroxymethyl)-3,4-piperidinediol (1:1)
(2S,3S)-2,3-Dihydroxysuccinic acid - (3R,4R,5R)-5-(hydroxymethyl)-3,4-piperidinediol (1:1)
(3R,4R,5R)-5-(Hydroxymethyl)-3,4-piperidinediol (2S,3S)-2,3-Dihydroxybutanedioate
Afegostat (D-Tartrate)

Biological Activity

[Description]:

Afegostat D-Tartrate is a pharmacological chaperone, which specifically and reversibly binds acid-β-glucosidase (GCase) in the endoplasmic reticulum (ER) with high affinity[1].

[Related Catalog]:

Signaling Pathways >> Others >> Others
Research Areas >> Neurological Disease

[Target]

GCase[1]


[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]. Richter F, et al. A GCase chaperone improves motor function in a mouse model of synucleinopathy. Neurotherapeutics. 2014 Oct;11(4):840-56.

Chemical & Physical Properties

[ Molecular Formula ]:
C10H19NO9

[ Molecular Weight ]:
297.26

[ Exact Mass ]:
297.105988

[ Storage condition ]:
2-8℃

Related Compounds