2-Methylsuccinic acid |
| カタログ番号GC31435 |
2-メチルコハク酸は、ヒトの体液中の正常な代謝産物であり、エチルマロン性脳症の主要な生化学的測定可能な特徴です。
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Cas No.: 498-21-5
Sample solution is provided at 25 µL, 10mM.
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2-Methylsuccinic acid is a normal metabolite in human fluids and the main biochemical measurable features in ethylmalonic encephalopathy.
2-Methylsuccinic acid is a normal metabolite in human fluids and the main biochemical measurable features in ethylmalonic encephalopathy[1]. The underlying biochemical defect involves isoleucine catabolism[2].
[1]. Grosso S, et al. Ethylmalonic encephalopathy: further clinical and neuroradiological characterization. J Neurol. 2002 Oct;249(10):1446-50. [2]. Nowaczyk MJ, et al. Ethylmalonic and methylsuccinic aciduria in ethylmalonic encephalopathy arise from abnormal isoleucine metabolism. Metabolism. 1998 Jul;47(7):836-9.
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