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Methylmalonic Acid-d3
本产品不向个人销售,仅用作科学研究,不用于任何人体实验及非科研性质的动物实验。
Methylmalonic Acid-d3图片
规格:98%
分子量:121.1
包装与价格:
包装价格(元)
5mg电议
10mg电议
25mg电议
50mg电议

产品介绍
甲基丙二酸-d3(甲基丙二酸-d3)是氘标记的甲基丙二酸。甲基丙二酸 (Methylmalonate) 是癌症中维生素 B-12 缺乏的指标。
货号:ajcx25894
CAS:42522-59-8
分子式:C4H3D3O4
分子量:121.1
溶解度:DMSO: slightly,Methanol: slightly
纯度:98%
存储:Store at -20°C
库存:现货

Background:


Methylmalonic acid-d3is intended for use as an internal standard for the quantification of methylmalonic acid by GC- or LC-MS. Methylmalonic acid is a dicarboxylic acid and byproduct of propionate catabolism.1It impairs mitochondrial respiration, increases glucose consumption, and downregulates the transcription of the mature neuronal markers neuron-specific enolase (ENO2) and synaptophysin (SYP) in neuronally differentiated SH-SY5Y cells. Intrastriatal administration of methylmalonic acid (100 mg/ml) induces neuronal loss in rats.2Methylmalonic acid accumulates in the tissues and body fluids of patients with methylmalonic acidemia, an inborn error of metabolism characterized by a deficiency in the activity of L-methylmaolnyl-CoA mutase or its cofactor 5-deoxyadenosylcobalamin and leads to seizures, intellectual disabilities, psychomotor abnormalities, and coma.1


1.da Costa, R.T., Dos Santos, M.B., Silva, I.C.S., et al.Methylmalonic acid compromises respiration and reduces the expression of differentiation markers of SH-SY5Y human neuroblastoma cellsACS Chem. Neurosci.12(14)2608-2618(2021) 2.Narasimhan, P., Sklar, R., Murrell, M., et al.Methylmalonyl-CoA mutase induction by cerebral ischemia and neurotoxicity of the mitochondrial toxin methylmalonic acidJ. Neurosci.16(22)7336-7346(1996)