추천 제품
제품명
N-Butyldeoxynojirimycin, film (dried in situ)
분석
≥98% (TLC)
Quality Level
양식
film (dried in situ)
solubility
water: 9.80-10.20 mg/mL, clear, colorless
저장 온도
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
유전자 정보
human ... UGCG(7357)
일반 설명
N-Butyldeoxynojirimycin is an alkylated product of imino sugar deoxynojirimycin.
애플리케이션
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.
생화학적/생리학적 작용
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.
Storage Class Code
11 - Combustible Solids
WGK
WGK 3
Flash Point (°F)
Not applicable
Flash Point (°C)
Not applicable
개인 보호 장비
Eyeshields, Gloves, type N95 (US)
이미 열람한 고객
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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