Conduritol B epoxide

产品说明书

Print
Chemical Structure| 6090-95-5 同义名 : D,L-1,2-Anhydro-myo-inositol;specific inhibitor of glucocerebrosidase in cultured cells;CBE;Activity: Inhibits alpha-glucosidase activity
CAS号 : 6090-95-5
货号 : A815375
分子式 : C6H10O5
纯度 : 98%+
分子量 : 162.141
MDL号 : MFCD00077326
存储条件：

粉末 Inert atmosphere,Store in freezer, under -20°C

液体 -20°C:3-6个月-80°C:12个月
溶解度 :

DMSO: 50 mg/mL(308.37 mM)，配合低频超声助溶，注意：DMSO长时间开封后，会吸水并导致溶解能力下降，请避免使用长期开封的DMSO

H2O: 100 mg/mL(616.75 mM)，配合低频超声助溶
动物实验配方：
生物活性
描述 Neuronopathic Gaucher disease is caused by mutations in GBA1 that encodes lysosomal acid β-glucosidase (GCase) that has glucosylceramide (GC) and its un-acylated form, glucosylsphingosine (GS) as substrates. Conduritol B epoxide (CBE) is an irreversible covalently bound GCase inhibitor. Differentiated N2a cells treated with CBE resulted in significant GC and GS accumulation in CBE-N2a cells, thereby creating a nGD model. CBE-N2a cells had higher calcium levels than in N2a cells without caffeine at baseline and higher cytosolic calcium levels compared to N2a cells. CBE-N2a cells showed a significant reduction in OCR (oxygen consumption rate) as evidenced by rate of ATP production, basal respiration and maximal respiration, compared to N2a cells, indicating reduced mitochondrial function in this nGD cell model[2]. In vivo, long-term daily CBE treatment (100 mg/kg) of 4L mice led to hind limb paralysis and small amounts of α-synuclein accumulation in the olfactory bulb and brainstem[3].
实验方案
1mg 5mg 10mg

1 mM

5 mM

10 mM

6.17mL

1.23mL

0.62mL

30.84mL

6.17mL

3.08mL

61.67mL

12.33mL

6.17mL
参考文献

[1]Liou B, Peng Y, et al. Modulating ryanodine receptors with dantrolene attenuates neuronopathic phenotype in Gaucher disease mice. Hum Mol Genet. 2016 Dec 1;25(23):5126-5141.

[2]Liou B, Peng Y, Li R, Inskeep V, Zhang W, Quinn B, Dasgupta N, Blackwood R, Setchell KD, Fleming S, Grabowski GA, Marshall J, Sun Y. Modulating ryanodine receptors with dantrolene attenuates neuronopathic phenotype in Gaucher disease mice. Hum Mol Genet. 2016 Dec 1;25(23):5126-5141

[3]Xu YH, Sun Y, Ran H, Quinn B, Witte D, Grabowski GA. Accumulation and distribution of α-synuclein and ubiquitin in the CNS of Gaucher disease mouse models. Mol Genet Metab. 2011 Apr;102(4):436-47