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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
CAS NO:42522-59-8
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。
Cas No.42522-59-8
别名甲基(D3)丙二酸,Methylpropanedioic acid-d3; Methylmalonate-d3
Canonical SMILESOC(C(C([2H])([2H])[2H])C(O)=O)=O
分子式C4H3D3O4
分子量121.1
溶解度DMSO: slightly,Methanol: slightly
储存条件-20°C
General tipsFor obtaining a higher solubility , please warm the tube at 37 ℃ and shake it in the ultrasonic bath for a while.
Shipping ConditionEvaluation sample solution : ship with blue ice
All other available size: ship with RT , or blue ice upon request
产品描述

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)