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Merck

B8299

Sigma-Aldrich

N-Butyldeoxynojirimycin

film (dried in situ), ≥98% (TLC)

Sinónimos:

Miglustat, NB-DNJ

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About This Item

Fórmula empírica (notación de Hill):
C10H21NO4
Número de CAS:
Peso molecular:
219.28
MDL number:
UNSPSC Code:
12352200
PubChem Substance ID:
NACRES:
NA.32

product name

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

Storage Class

11 - Combustible Solids

wgk_germany

WGK 3

flash_point_f

Not applicable

flash_point_c

Not applicable

ppe

Eyeshields, Gloves, type N95 (US)


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Olivier Bonnot et al.
The world journal of biological psychiatry : the official journal of the World Federation of Societies of Biological Psychiatry, 20(4), 310-319 (2017-09-16)
Objectives: Niemann-Pick disease type C (NP-C) is a rare inherited neurovisceral disease that should be recognised by psychiatrists as a possible underlying cause of psychiatric abnormalities. This study describes NP-C patients who had psychiatric manifestations at enrolment in the international
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
N-butyldeoxynojirimycin is a novel inhibitor of glycolipid biosynthesis.
Platt FM et al.
The Journal of Biological Chemistry, 269(11), 8362-8365 (1994)
How adhesion/growth-regulatory galectins-1 and-3 attain cell specificity: case study defining their target on neuroblastoma cells (SK-N-MC) and marked affinity regulation by affecting microdomain organization of the membrane
Kopitz J, et al.
IUBMB Life, 62(8), 624-628 (2010)
Imiglucerase in the treatment of Gaucher disease: a history and perspective
Deegan PB and Cox TM
Drug design, development and therapy, 6, 81-81 (2012)

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