上海莼试生物技术有限公司
上海莼试生物技术有限公司
化学性质：
| 规格 | 25 mg 50 mg 100 mg 250 mg |
| CAS | 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 tips | For obtaining a higher solubility , please warm the tube at 37 ℃ and shake it in the ultrasonic bath for a while. |
| Shipping Condition | Evaluation sample solution : ship with blue ice |
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.2,1
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)
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