B8299
N-Butyldeoxynojirimycin
film (dried in situ), ≥98% (TLC)
동의어(들):
Miglustat, NB-DNJ
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제품정보 (DICE 배송 시 비용 별도)
실험식(Hill 표기법):
C10H21NO4
CAS 번호:
Molecular Weight:
219.28
UNSPSC Code:
12352200
PubChem Substance ID:
NACRES:
NA.32
MDL number:
제품 이름
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
저장 등급
11 - Combustible Solids
wgk
WGK 3
flash_point_f
Not applicable
flash_point_c
Not applicable
ppe
Eyeshields, Gloves, type N95 (US)
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
국제 무역 품목 번호
| SKU | GTIN |
|---|---|
| B8299-1MG | 04061826740996 |
| B8299-5MG | 04061833627846 |