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About This Item
经验公式（希尔记法）:
C10H21NO4
CAS号:
分子量:
219.28
MDL號碼:
分類程式碼代碼:
12352200
PubChem物質ID:
NACRES:
NA.32
化驗
≥98% (TLC)
品質等級
形狀
film (dried in situ)
溶解度
water: 9.80-10.20 mg/mL, clear, colorless
儲存溫度
2-8°C
SMILES 字串
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 密鑰
UQRORFVVSGFNRO-UTINFBMNSA-N
基因資訊
human ... UGCG(7357)
一般說明
N-Butyldeoxynojirimycin is an alkylated product of imino sugar deoxynojirimycin.
應用
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.
生化／生理作用
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
儲存類別代碼
11 - Combustible Solids
水污染物質分類（WGK）
WGK 3
閃點（°F）
Not applicable
閃點（°C）
Not applicable
個人防護裝備
Eyeshields, Gloves, type N95 (US)
其他客户在看
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
Frances M Platt et al.
Nature reviews. Disease primers, 4(1), 27-27 (2018-10-03)
Lysosomal storage diseases (LSDs) are a group of over 70 diseases that are characterized by lysosomal dysfunction, most of which are inherited as autosomal recessive traits. These disorders are individually rare but collectively affect 1 in 5,000 live births. LSDs
Arunabha Ghosh et al.
Orphanet journal of rare diseases, 12(1), 117-117 (2017-06-28)
Mucopolysaccharidosis type III is a progressive, neurodegenerative lysosomal storage disorder for which there is currently no effective therapy. Though numerous potential therapies are in development, there are several challenges to conducting clinical research in this area. We seek to make
Cytoskeletal inhibitors, anti-adhesion molecule antibodies, and lectins inhibit hepatocyte spheroid formation
Nakamura M, et al.
Acta Medica Okayama, 56(1), 43-50 (2002)
The role of the iminosugar N-butyldeoxynojirimycin (miglustat) in the management of type I (non-neuronopathic) Gaucher disease: a position statement
Cox TM, et al.
Journal of inherited metabolic disease, 26(6), 513-526 (2003)
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