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B8299

Sigma-Aldrich

N-Butyldeoxynojirimycin

film (dried in situ)

Sinonimo/i:

Miglustat, NB-DNJ

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About This Item

Formula empirica (notazione di Hill):
C10H21NO4
Numero CAS:
Peso molecolare:
219.28
Numero MDL:
Codice UNSPSC:
12352200
ID PubChem:
NACRES:
NA.32

Saggio

≥98% (TLC)

Livello qualitativo

Forma fisica

film (dried in situ)

Solubilità

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

Temperatura di conservazione

2-8°C

Stringa SMILE

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
UQRORFVVSGFNRO-UTINFBMNSA-N

Informazioni sul gene

human ... UGCG(7357)

Categorie correlate

Descrizione generale

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

Applicazioni

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.

Azioni biochim/fisiol

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

Codice della classe di stoccaggio

11 - Combustible Solids

Classe di pericolosità dell'acqua (WGK)

WGK 3

Punto d’infiammabilità (°F)

Not applicable

Punto d’infiammabilità (°C)

Not applicable

Dispositivi di protezione individuale

Eyeshields, Gloves, type N95 (US)


Certificati d'analisi (COA)

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Medicine, 96(45), e8492-e8492 (2017-11-16)
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Nature reviews. Disease primers, 4(1), 27-27 (2018-10-03)
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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
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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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