Afegostat (D-Isofagomine) (Synonyms: D-Isofagomine; Isofagomine) |
| カタログ番号GC33728 |
アフェゴスタット (D-イソファゴミン) は、小胞体 (ER) の酸-β-グルコシダーゼ (GCase) に特異的かつ可逆的に結合する薬理学的シャペロンです。
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Cas No.: 169105-89-9
Sample solution is provided at 25 µL, 10mM.
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Afegostat is a pharmacological chaperone, which specifically and reversibly binds acid-β-glucosidase (GCase) in the endoplasmic reticulum (ER) with high affinity[1].
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].
[1]. Richter F, et al. A GCase chaperone improves motor function in a mouse model of synucleinopathy. Neurotherapeutics. 2014 Oct;11(4):840-56.
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