N-BUTYLDEOXYNOJIRIMYCIN

N-BUTYLDEOXYNOJIRIMYCIN 속성

N-BUTYLDEOXYNOJIRIMYCIN C화학적 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.

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-BUTYLDEOXYNOJIRIMYCIN 준비 용품 및 원자재

원자재

준비 용품

N-BUTYLDEOXYNOJIRIMYCIN 공급 업체

공급자	전화	이메일	국가	제품 수	이점
Hebei Mojin Biotechnology Co., Ltd	+8613288715578	sales@hbmojin.com	China	12456	58
Shanghai Daken Advanced Materials Co.,Ltd	+86-371-66670886	info@dakenam.com	China	15928	58
Hangzhou FandaChem Co.,Ltd.	008657128800458; +8615858145714	fandachem@gmail.com	China	9348	55
Shanghai Zheyan Biotech Co., Ltd.	18017610038	zheyansh@163.com	CHINA	3620	58
career henan chemical co	+86-0371-86658258	sales@coreychem.com	China	29914	58
Chengdu Biopurify Phytochemicals Ltd.	+8618080483897	sales@biopurify.com	China	3424	58
Hebei Guanlang Biotechnology Co., Ltd.	+86-19930503282	alice@crovellbio.com	China	8823	58
BOC Sciences	+1-631-485-4226	inquiry@bocsci.com	United States	19553	58
Chongqing Chemdad Co., Ltd	+86-023-61398051 +8613650506873	sales@chemdad.com	China	39916	58
TargetMol Chemicals Inc.	+1-781-999-5354 +1-00000000000	marketing@targetmol.com	United States	19892	58

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