2-Methylsuccinic acid

本公司产品仅用于科研实验，不得用于人体或动物的临床与诊断

甲基琥珀酸是人体体液中的正常代谢物。尿液中甲基琥珀酸（连同乙基丙二酸）水平升高是乙基丙二酸脑病的主要生化可测量特征，乙基丙二酸脑病是一种罕见的代谢性疾病，具有常染色体隐性遗传模式，临床特征为神经运动迟缓、高乳酸血症、复发性瘀点、体位性手足发绀、和慢性腹泻。

Methylsuccinic acid is a normal metabolite found in human fluids. Increased urinary levels of Methylsuccinic acid (together with ethylmalonic acid) are the main biochemical measurable features in ethylmalonic encephalopathy a rare metabolic disorder with an autosomal recessive mode of inheritance that is clinically characterized by neuromotor delay hyperlactic acidemia recurrent petechiae orthostatic acrocyanosis and chronic diarrhea.

2-Methylsuccinic acid is a normal metabolite in human fluids and the main biochemical measurable features in ethylmalonic encephalopathy. The underlying biochemical defect involves isoleucine catabolism.

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Methylbutanedioic acid | Pyrotartaric Acid

Others

Other Targets

Others

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498-21-5

132.11

C5H8O4

>98% (HPLC)

DMSO:200 mM

CC(CC(O)=O)C(O)=O

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(-20℃)

With Ice Pack

≥ 2 years