
2-Methylsuccinic acid
CAS No. 498-21-5
2-Methylsuccinic acid ( Methylbutanedioic acid | Pyrotartaric Acid )
产品货号. M19998 CAS No. 498-21-5
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。
纯度: >98% (HPLC)






规格 | 价格/人民币 | 库存 | 数量 |
500MG | ¥332 | 有现货 |
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1G | ¥389 | 有现货 |
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生物学信息
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产品名称2-Methylsuccinic acid
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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
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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。
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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.
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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.
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体内实验——
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同义词Methylbutanedioic acid | Pyrotartaric Acid
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通路Others
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靶点Other Targets
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受体Others
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研究领域——
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适应症——
化学信息
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CAS Number498-21-5
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分子量132.11
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分子式C5H8O4
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纯度>98% (HPLC)
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溶解度DMSO:200 mM
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SMILESCC(CC(O)=O)C(O)=O
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化学全称——
运输与储存
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储存条件(-20℃)
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运输条件With Ice Pack
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稳定性≥ 2 years
参考文献
1.Grosso S et al. Ethylmalonic encephalopathy: further clinical and neuroradiological characterization. J Neurol. 2002 Oct;249(10):1446-50.
产品手册




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