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3-Methylglutaconic Acid
本产品不向个人销售,仅用作科学研究,不用于任何人体实验及非科研性质的动物实验。
3-Methylglutaconic Acid图片
CAS NO:5746-90-7
包装与价格:
包装价格(元)
25mg电议
50mg电议
100mg电议
250mg电议

产品介绍
A branched-chain organic acid
Cas No.5746-90-7
别名3-甲基戊烯二酸
Canonical SMILESO=C(C=C(CC(O)=O)C)O
分子式C6H8O4
分子量144.1
溶解度DMF: 30 mg/ml,DMSO: 30 mg/ml,Ethanol: 30 mg/ml,PBS (pH 7.2): 10 mg/ml
储存条件-20°C
General tipsFor obtaining a higher solubility , please warm the tube at 37 ℃ and shake it in the ultrasonic bath for a while.
Shipping ConditionEvaluation sample solution : ship with blue ice
All other available size: ship with RT , or blue ice upon request
产品描述

3-Methylglutaconic acid (3-MGA) is a branched-chain organic acid and product of leucine catabolism.13-MGA accumulates in 3-methylglutaconic aciduria, which is caused by an inborn error of leucine metabolism characterized by 3-MGA-CoA hydratase deficiency, leading to delayed development, hypoglycemia, metabolic acidosis, and neurological impairments.2,1

1.Wortmann, S.B., Kluijtmans, L.A., Engelke, U.F.H., et al.The 3-methylglutaconic acidurias: What’s new•J. Inherit. Metab. Dis.35(1)13-22(2012) 2.Leipnitz, G., Seminotti, B., Amaral, A.U., et al.Induction of oxidative stress by the metabolites accumulating in 3-methylglutaconic aciduria in cerebral cortex of young ratsLife Sci.82(11-12)652-662(2008)