2-Methylsuccinic acid ( Methylbutanedioic acid | Pyrotartaric Acid )

产品货号. M19998 CAS No. 498-21-5

COA

Datasheet

HNMR

HPLC

MSDS

Handing Instructions

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产品名称

2-Methylsuccinic acid
注意事项

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
产品简述

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。
产品描述

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.
体外实验

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.
体内实验

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同义词

Methylbutanedioic acid | Pyrotartaric Acid
通路

Others
靶点

Other Targets
受体

Others
研究领域

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适应症

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1.Grosso S et al. Ethylmalonic encephalopathy: further clinical and neuroradiological characterization. J Neurol. 2002 Oct;249(10):1446-50.

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