|(2R,3R,4R,5S)-1-丁基-2-(羟甲基)哌啶-3,4,5-三醇盐酸盐|CsnPharm

CAS No.: 210110-90-0

Synonyms: N-Butyldeoxynojirimycin hydrochloride;NB-DNJ hydrochloride;OGT 918 hydrochloride

Miglustat HCl is an inhibitor of glucosylceramide synthase, primarily to treat Type I Gaucher disease (GD1).

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400-920-2911

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400-920-2911

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描述Gaucher disease (GD) is an inherited lipidstorage disorder caused by deficiency of glucocerebrosidase (GlcCerase) activity and characterized by intralysosomal accumulation of glucocerebroside (glucosylceramide; GlcCer) that leads to dysfunction in multiple organ systems. Miglustat is an iminosugar that reversibly inhibits glucosylceramide synthase and reduces GlcCer biosynthesis^[3]. The addition of 10 μM miglustat to COS-7 cell medium leads to 1.3-, 2.1-, 2.3-, 3.6-, and 9.9-fold increase in the activity of S364R, wild-type, N370S, V15M, and M123T GC (β-glucosidase), respectively. These results suggest that miglustat, in addition to the inhibitory effect on CSG (glucosyltransferase), also works as a "chemical chaperone", increasing the activity of acid beta-glucosidase of wild-type and several GC mutated proteins, including the most frequent N370S mutation^[4].
作用机制Miglustat binds at the active site^[4].
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技术信息

CAS号	210110-90-0	储存条件	粉末 Inert atmosphere,Room Temperature 液体 -20°C:3-6 月 -80°C:12月
分子式	C10H22ClNO4	运输	蓝冰
分子量	255.74	别名	N-Butyldeoxynojirimycin hydrochloride;NB-DNJ hydrochloride;OGT 918 hydrochloride;N-Butyldeoxynojirimycin HCl;OGT-918 HCl;N-Butyldeoxynojirimycin (hydrochloride);Miglustat;NB-DNJ;OGT-918;N-butyldeoxynojirimycin;Brand name: Zavesca

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