3-Methylglutaconic Acid

A branched-chain organic acid

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库存: 现货

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25mg

￥1425.00

1140.00

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50mg

￥2425.00

1940.00

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100mg

￥4237.00

3390.00

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250mg

￥9225.00

7380.00

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货号: ajcx25276
CAS: 5746-90-7
别名: 3-甲基戊烯二酸
分子式: C6H8O4
分子量: 144.1
纯度: >98%
溶解度: DMF: 30 mg/ml,DMSO: 30 mg/ml,Ethanol: 30 mg/ml,PBS (pH 7.2): 10 mg/ml
储存: -20°C
库存: 现货

Background

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)