A branched-chain organic acid
货号:ajcx25276
CAS:5746-90-7
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
纯度：98%
存储：Store at -20°C
库存：现货

Background:

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)