N-Butyldeoxynojirimycin Chemical Properties

Melting point:

126-127℃

alpha 

D25 -15.9° (c = 0.77 in water)

Boiling point:

394.7±42.0 °C(Predicted)

Density 

1.234

RTECS 

TN4350150

storage temp. 

2-8°C

solubility 

DMSO 44 mg/mL (200.66 mM) Ethanol 22 mg/mL (100.33 mM) Water 44 mg/mL (200.66 mM)

pka

13.72±0.70(Predicted)

form 

Powder

color 

White

Water Solubility 

Soluble in water at 10mg/ml

InChI

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

InChIKey

UQRORFVVSGFNRO-UTINFBMNSA-N

SMILES

N1(CCCC)C[C@H](O)[C@@H](O)[C@H](O)[C@H]1CO

Safety Information

WGK Germany 

3

HS Code 

2933399990

MSDS

• Language:English Provider:SigmaAldrich

N-Butyldeoxynojirimycin Usage And Synthesis

Description

Miglustat is an N-alkylated iminosugar, launched as an oral treatment for mild to moderate type 1 Gaucher’s disease in adult patients for whom enzyme replacement therapy is not a therapeutic option. It is readily synthesized from D-glucose in three steps by first converting to N-butylglucamine via reductive amination with butylamine, followed by a microbial oxidation to an aminofuranose intermediate and subsequent reductive cyclization. Type 1 Gaucher’s disease is a metabolic disorder caused by the lysosomal accumulation of certain glycosphingolipids (GSLs) as a result of deficiency in their degradation. Enlargement of the liver and spleen, low blood platelet and bone lesions are among the key symptoms of this disease. Miglustat acts by inhibiting glucosylceramide synthase, a glucosyl transferase enzyme in the biosynthesis of most GSLs, which results in the lowering of GSLs to a level that can be effectively cleared. Up to 50% reduction in liver and splenocyte GSL levels are achieved in mice by long-term administration of Miglustat (600– 1800 mg/kg/day for 118 days). Miglustat, dosed at 50 and 100 mg in Gaucher patients, exhibits dose proportionate pharmacokinetics (t_max=2.5 h, t_1/2=6 to 7 h) and ＞90% oral bioavailability. Steady-state plasma levels are reached after 4–6 weeks of treatment. Miglustat is not significantly metabolized in humans and the major route of excretion is renal. In clinical trials, efficacy was demonstrated by significant reductions in liver and spleen volumes (12 and 19%, respectively) at 12 months and increase in hemoglobin and platelet count (0.91 g/dL and 13.6×10⁹/l, respectively) at 24 months. Miglustat is generally well tolerated by patients and the most common side effects are diarrhea and weight loss.

Originator

G.D. Searle (Pfizer) (US)

Uses

An alpha-glucosidase Inhibitor

Uses

Treatment of glycolipid storage diseases.

Definition

ChEBI: A hydroxypiperidine that is deoxynojirimycin in which the amino hydrogen is replaced by a butyl group.

Indications

N-butyldeoxynojirimycin is an approved drug for Niemann–Pick disease type C and Gaucher disease type 1, which are lipid storage disorders[1].

brand name

Zavesca(Actelion);Vevesca.

General Description

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

Biological Activity

Orally active α -glucosidase I and II and ceramide-specific glycosyltransferase inhibitor. Rescues trafficking-deficient F508del-CTFR in human airway epithelial cells via inhibition of ER α -glucosidases I and II. Also has broad spectrum antiviral activity.

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.

References

[1] Hiroyuki Nakamura . “N-butyldeoxynojirimycin (miglustat) ameliorates pulmonary fibrosis through inhibition of nuclear translocation of Smad2/3.” Biomedicine & Pharmacotherapy 160 (2023): Article 114405.

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