CAS:6090-95-5Conduritol B Epoxide

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Synonyms: Conduritol-B Epoxide

Conduritol B epoxide is an irreversible inhibitor of acid β-glucosidase (GCase) with IC50 of 1 μM.

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18817579266

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18817579266

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实验数据

技术信息

CAS号

6090-95-5

储存条件

分子式

C6H10O5

运输

蓝冰

分子量

162.14

别名

Conduritol-B Epoxide

溶解度

DMSO	195.0 mg/mL (1202.7 mM)
Water	140.0 mg/mL (863.4 mM)

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].