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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:
72599-27-0
Peso molecular:
219.28
MDL number:
MFCD00272581
UNSPSC Code:
12352200
PubChem Substance ID:
24892070
NACRES:
NA.32

product name

N-Butyldeoxynojirimycin, film (dried in situ)

assay

≥98% (TLC)

Quality Level

200

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)

Categorías relacionadas

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)

Certificados de análisis (COA)

Busque Certificados de análisis (COA) introduciendo el número de lote del producto. Los números de lote se encuentran en la etiqueta del producto después de las palabras «Lot» o «Batch»

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Acarbose ≥95% (HPLC)

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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)
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
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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