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Methylmalonic Acid-d3 Sale

(Synonyms: 甲基(D3)丙二酸,Methylpropanedioic acid-d3; Methylmalonate-d3) 目录号 : GC49472

An internal standard for the quantification of methylmalonic acid

Methylmalonic Acid-d3 Chemical Structure

Cas No.:42522-59-8

规格 价格 库存 购买数量
5mg
¥1,096.00
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10mg
¥1,976.00
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25mg
¥4,384.00
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50mg
¥7,671.00
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Sample solution is provided at 25 µL, 10mM.

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产品描述

Methylmalonic acid-d3 is 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.1 It 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.2 Methylmalonic 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)

Chemical Properties

Cas No. 42522-59-8 SDF Download SDF
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
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储备液的保存方式和期限:-80°C 储存时,请在 6 个月内使用,-20°C 储存时,请在 1 个月内使用。
为了提高溶解度,请将管子加热至37℃,然后在超声波浴中震荡一段时间。
Shipping Condition 评估样品解决方案:配备蓝冰进行发货。所有其他可用尺寸:配备RT,或根据请求配备蓝冰。

溶解性数据

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1 mg 5 mg 10 mg
1 mM 8.2576 mL 41.2882 mL 82.5764 mL
5 mM 1.6515 mL 8.2576 mL 16.5153 mL
10 mM 0.8258 mL 4.1288 mL 8.2576 mL
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