N-부틸데옥시노지리마이신

한글명:N-부틸데옥시노지리마이신

카스 번호72599-27-0

상품명:N-Butyldeoxynojirimycin

CBNumberCB0125257

분자식C10H21NO4

포뮬러 무게219.28

MOL 파일72599-27-0.mol

동의어(한글)

N-부틸데옥시노지리마이신

화학적 성질

녹는점	126-127℃
알파	D25 -15.9° (c = 0.77 in water)
끓는 점	394.7±42.0 °C(Predicted)
밀도	1.234
RTECS 번호	TN4350150
저장 조건	2-8°C
용해도	DMSO 44 mg/mL (200.66 mM) Ethanol 22 mg/mL (100.33 mM) Water 44 mg/mL (200.66 mM)
물리적 상태	가루
산도 계수 (pKa)	13.72±0.70(Predicted)
색상	하얀색
수용성	10mg/ml의 물에 용해됨
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

위험 및 안전 성명

WGK 독일	3
HS 번호	2933399990

그림문자(GHS)

N-부틸데옥시노지리마이신 화학적 특성, 용도, 생산

개요
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.
용도
Treatment of glycolipid storage diseases.
정의
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].
일반 설명
N-Butyldeoxynojirimycin is an alkylated product of imino sugar deoxynojirimycin.
생물학적 활성
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.

N-부틸데옥시노지리마이신 공급 업체

N-부틸데옥시노지리마이신 관련 검색

Send Inquriy