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产品属性：
产品名称 | 3-Methylglutaconic Acid |
规格 | 25 mg、50 mg、100 mg、250 mg |
货号 | EY-01Y16817 |
Cas No.: 5746-90-7
别名: N/A
化学名: N/A
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.1 3-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.2,11.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)
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