3-Methylglutaconic Acid (Synonyms: β-Methylglutaconic Acid, 3-MGA, 3-MGC Acid, NSC 249232) |
| Catalog No.GC49149 |
A branched-chain organic acid
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Cas No.: 5746-90-7
Sample solution is provided at 25 µL, 10mM.
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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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