Product Description: 2-Methylsuccinic acid-d6 is the deuterium labeled 2-Methylsuccinic acid[1]. 2-Methylsuccinic acid is a normal metabolite in human fluids and the main biochemical measurable features in ethylmalonic encephalopathy[2][3].
Formula: C5H2D6O4
References: [1]Russak EM, et al. Impact of Deuterium Substitution on the Pharmacokinetics of Pharmaceuticals. Ann Pharmacother. 2019 Feb;53(2):211-216./[2]Grosso S, et al. Ethylmalonic encephalopathy: further clinical and neuroradiological characterization. J Neurol. 2002 Oct;249(10):1446-50./[3]Nowaczyk MJ, et al. Ethylmalonic and methylsuccinic aciduria in ethylmalonic encephalopathy arise from abnormal isoleucine metabolism. Metabolism. 1998 Jul47(7):836-9.