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Merck
CN

B8299

N-Butyldeoxynojirimycin

film (dried in situ), ≥98% (TLC)

别名:

Miglustat, NB-DNJ

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关于此项目

经验公式(希尔记法):
C10H21NO4
化学文摘社编号:
分子量:
219.28
UNSPSC Code:
12352200
PubChem Substance ID:
NACRES:
NA.32
MDL number:
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产品名称

N-Butyldeoxynojirimycin, film (dried in situ)

assay

≥98% (TLC)

Quality Level

form

film (dried in situ)

solubility

water: 9.80-10.20 mg/mL, clear, colorless

storage temp.

2-8°C

SMILES string

CCCCN1C[C@H](O)[C@@H](O)[C@H](O)[C@H]1CO

InChI

1S/C10H21NO4/c1-2-3-4-11-5-8(13)10(15)9(14)7(11)6-12/h7-10,12-15H,2-6H2,1H3/t7-,8+,9-,10-/m1/s1

InChI key

UQRORFVVSGFNRO-UTINFBMNSA-N

Gene Information

human ... UGCG(7357)

General description

N-Butyldeoxynojirimycin is an alkylated product of imino sugar deoxynojirimycin.

Application

N-Butyldeoxynojirimycin has been used:
  • in the inhibition of glycolipid synthesis in neuroblastoma cells
  • in the inhibition the ceramide-specific glycosyltransferase in hepatocytes
  • in the inhibition of β-glucosidase (GBA2) using fluorescence- activity assay in human embryonic kidney (HEK293) cells.

Biochem/physiol Actions

N-Butyldeoxynojirimycin is an inhibitor of glucosyltransferase and α-glucosidases. N-Butyldeoxynojirimycin, also known as misglustat, reduces glycolipid levels by substrate reduction therapy (SRT) and is effectively used for the treatment of glycosphingolipid lysosomal storage disorder, Gaucher disease.
α-glucosidase Inhibitor


存储类别

11 - Combustible Solids

wgk

WGK 3

flash_point_f

Not applicable

flash_point_c

Not applicable

ppe

Eyeshields, Gloves, type N95 (US)



历史批次信息供参考:

分析证书(COA)

Lot/Batch Number

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N-butyldeoxynojirimycin is a novel inhibitor of glycolipid biosynthesis.
Platt FM et al.
The Journal of Biological Chemistry, 269(11), 8362-8365 (1994)
Maciej Machaczka et al.
Upsala journal of medical sciences, 117(1), 28-34 (2012-01-18)
Gaucher disease (GD) is an infrequent progressive multisystem lysosomal storage disorder caused by the deficient activity of the lysosomal enzyme, glucocerebrosidase. A retrospective, single-center analysis of the clinical experience concerning the use of miglustat (N-butyldeoxynojirimycin), an oral inhibitor of glucosylceramide
Aizeddin Mhanni et al.
Diagnostics (Basel, Switzerland), 10(2) (2020-01-30)
Intraocular lesions have been infrequently reported in patients with Gaucher disease type 3 (GD3). We previously reported siblings with GD3 who responded well to the combination of enzyme replacement therapy (ERT) and substrate reduction therapy (SRT). Here we report progressive



全球贸易项目编号

货号GTIN
B8299-1MG04061826740996
B8299-5MG04061833627846