Methylmalonyl-Coenzyme A (sodium salt)
(Synonyms: Methylmalonyl-CoA) 目录号 : GC47664
An intermediate in metabolic pathways
Sample solution is provided at 25 µL, 10mM.
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Quality Control & SDS
- View current batch:
- Purity: >95.00%
- COA (Certificate Of Analysis)
- SDS (Safety Data Sheet)
- Datasheet
Methylmalonyl coenzyme A (methylmalonyl-CoA) is an intermediate in multiple metabolic pathways in bacteria and eukaryotes.1,2,3 It is an intermediate in carbon assimilation in certain bacteria and carbon fixation in plants.1,2 Methylmalonyl-CoA is converted to succinyl-CoA by methylmalonyl-CoA mutase with vitamin B12 as a coenzyme.3 A deficiency in vitamin B12 leads to a build-up of methylmalonyl-CoA.4
1.Anthony, C.How half a century of research was required to understand bacterial growth on C1 and C2 compounds; the story of the serine cycle and the ethylmalonyl-CoA pathwaySci. Prog.94(Pt 2)109-137(2011) 2.Tabita, F.R.The hydroxypropionate pathway of CO2 fixation: Fait accompliProc. Natl. Acad. Sci. U.S.A.106(50)21015-21016(2009) 3.Medicine, I.o.Vitamin B12Dietary reference intakes for thiamin, riboflavin, niacin, vitamin B6, folate, vitamin B12, pantothenic acid, biotin, and choline306-356(1998) 4.Cardinale, G.J., Carty, T.J., and Abeles, R.H.Effect of methylmalonyl coenzyme A, a metabolite which accumulates in vitamin B12 deficiency, on fatty acid synthesisJ. Biol. Chem.245(15)3771-3775(1970)
| Cas No. | N/A | SDF | |
| 别名 | Methylmalonyl-CoA | ||
| Canonical SMILES | O[C@H]1[C@H](N2C=NC3=C2N=CN=C3N)O[C@H](COP(OP(OCC(C)(C)[C@@H](O)C(NCCC(NCCSC(C(C(O)=O)C)=O)=O)=O)(O)=O)(O)=O)[C@H]1OP(O)(O)=O.[Na] | ||
| 分子式 | C25H40N7O19P3S.XNa | 分子量 | 867.6 |
| 溶解度 | PBS (pH 7.2): 10 mg/ml | 储存条件 | Store at -20°C |
| General tips | 请根据产品在不同溶剂中的溶解度选择合适的溶剂配制储备液；一旦配成溶液，请分装保存，避免反复冻融造成的产品失效。 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。 |
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| 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。 | ||
| 制备储备液 | |||
 |
1 mg | 5 mg | 10 mg |
| 1 mM | 1.1526 mL | 5.763 mL | 11.526 mL |
| 5 mM | 0.2305 mL | 1.1526 mL | 2.3052 mL |
| 10 mM | 0.1153 mL | 0.5763 mL | 1.1526 mL |
| 第一步：请输入基本实验信息（考虑到实验过程中的损耗，建议多配一只动物的药量） | ||||||||||
| 给药剂量 | mg/kg |  |
动物平均体重 | g | |
每只动物给药体积 | ul | |
动物数量 | 只 |
| 第二步：请输入动物体内配方组成（配方适用于不溶于水的药物；不同批次药物配方比例不同，请联系GLPBIO为您提供正确的澄清溶液配方） | ||||||||||
| % DMSO % % Tween 80 % saline | ||||||||||
| 计算重置 | ||||||||||
计算结果:
工作液浓度： mg/ml；
DMSO母液配制方法： mg 药物溶于 μL DMSO溶液（母液浓度 mg/mL，
体内配方配制方法：取 μL DMSO母液，加入 μL PEG300，混匀澄清后加入μL Tween 80，混匀澄清后加入 μL saline，混匀澄清。
1. 首先保证母液是澄清的；
2.
一定要按照顺序依次将溶剂加入，进行下一步操作之前必须保证上一步操作得到的是澄清的溶液，可采用涡旋、超声或水浴加热等物理方法助溶。
3. 以上所有助溶剂都可在 GlpBio 网站选购。
Identification of 2,2-Dimethylbutanoic Acid (HST5040), a Clinical Development Candidate for the Treatment of Propionic Acidemia and Methylmalonic Acidemia
J Med Chem 2021 Apr 22;64(8):5037-5048.PMID:33848153DOI:10.1021/acs.jmedchem.1c00124.
Propionic acidemia (PA) and methylmalonic acidemia (MMA) are rare autosomal recessive disorders of propionyl-CoA (P-CoA) catabolism, caused by a deficiency in the enzymes P-CoA carboxylase and methylmalonyl-CoA (M-CoA) mutase, respectively. PA and MMA are classified as intoxication-type inborn errors of metabolism because the intramitochondrial accumulation of P-CoA, M-CoA, and other metabolites results in secondary inhibition of multiple pathways of intermediary metabolism, leading to organ dysfunction and failure. Herein, we describe the structure-activity relationships of a series of short-chain carboxylic acids which reduce disease-related metabolites in PA and MMA primary hepatocyte disease models. These studies culminated in the identification of 2,2-dimethylbutanoic acid (10, HST5040) as a clinical candidate for the treatment of PA and MMA. Additionally, we describe the in vitro and in vivo absorption, distribution, metabolism, and excretion profile of HST5040, data from preclinical studies, and the synthesis of the sodium salt of HST5040 for clinical trials.