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:
产品名称
N-Butyldeoxynojirimycin, film (dried in situ)
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
assay
≥98% (TLC)
form
film (dried in situ)
solubility
water: 9.80-10.20 mg/mL, clear, colorless
storage temp.
2-8°C
Quality Level
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
α-glucosidase Inhibitor
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.
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)
N-butyldeoxynojirimycin is a novel inhibitor of glycolipid biosynthesis.
Platt FM et al.
The Journal of Biological Chemistry, 269(11), 8362-8365 (1994)
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
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
Andrés Felipe Leal et al.
International journal of molecular sciences, 21(17) (2020-09-02)
GM2 gangliosidoses are a group of pathologies characterized by GM2 ganglioside accumulation into the lysosome due to mutations on the genes encoding for the β-hexosaminidases subunits or the GM2 activator protein. Three GM2 gangliosidoses have been described: Tay-Sachs disease, Sandhoff
Beom Hee Lee et al.
Medicine, 96(45), e8492-e8492 (2017-11-16)
Gaucher disease (GD) is caused by a deficiency in the lysosomal enzyme glucocerebrosidase. Enzyme replacement therapy (ERT) is recommended for clinical improvement. The efficacy and safety of a new imiglucerase, Abcertin, were assessed in 7 Egyptian patients with treatment-naïve type
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