CAS NO: | 42522-59-8 |
包装 | 价格(元) |
5mg | 电议 |
10mg | 电议 |
25mg | 电议 |
50mg | 电议 |
Cas No. | 42522-59-8 |
别名 | 甲基(D3)丙二酸,Methylpropanedioic acid-d3; Methylmalonate-d3 |
Canonical SMILES | OC(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 tips | For obtaining a higher solubility , please warm the tube at 37 ℃ and shake it in the ultrasonic bath for a while. |
Shipping Condition | Evaluation 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) |