Medium-Chain Specific Acyl-CoA Dehydrogenase (ACADM) is a mitochondrial fatty acid beta-oxidation that belongs to the acyl-CoA dehydrogenase family. ACADM is a homotetramer enzyme that catalyzes the initial step of the mitochondrial fatty acid beta-oxidation pathway. ACADM is specific for acyl chain lengths of 4 to 16. It is essential for converting these particular fatty acids to energy, especially during fasting periods. Defects in ACADM cause medium-chain acyl-CoA dehydrogenase deficiency, a disease characterized by hepatic dysfunction, fasting hypoglycemia, and encephalopathy, which can result in infantile death.
规格 | 价格/CNY | 货期 | 数量 | |
---|---|---|---|---|
10 μg | ¥ 820 | 5日内发货 | ||
50 μg | ¥ 2,480 | 5日内发货 | ||
500 μg | ¥ 12,100 | 5日内发货 | ||
1 mg | ¥ 17,400 | 5日内发货 |
产品描述 | Medium-Chain Specific Acyl-CoA Dehydrogenase (ACADM) is a mitochondrial fatty acid beta-oxidation that belongs to the acyl-CoA dehydrogenase family. ACADM is a homotetramer enzyme that catalyzes the initial step of the mitochondrial fatty acid beta-oxidation pathway. ACADM is specific for acyl chain lengths of 4 to 16. It is essential for converting these particular fatty acids to energy, especially during fasting periods. Defects in ACADM cause medium-chain acyl-CoA dehydrogenase deficiency, a disease characterized by hepatic dysfunction, fasting hypoglycemia, and encephalopathy, which can result in infantile death. |
种属 | Human |
表达系统 | E. coli |
标签 | N-6His |
蛋白编号 | P11310 |
别名 | ACADM, Medium-Chain Specific Acyl-CoA Dehydrogenase Mitochondrial, MCAD |
氨基酸序列 | Lys26-Asn421 |
蛋白构建 | Recombinant Human Medium-Chain Specific Acyl-CoA Dehydrogenase, Mitochondrial is produced by our E.coli expression system and the target gene encoding Lys26-Asn421 is expressed with a 6His tag at the N-terminus. |
蛋白纯度 | Greater than 95% as determined by reducing SDS-PAGE. (QC verified) |
分子量 | 42 KDa, reducing conditions |
内毒素 | Less than 1 EU/µg as determined by LAL test. |
缓冲液 | Supplied as a 0.2 μm filtered solution of 20mM Acetate, 10% Trehalose, 0.05% Tween 80, pH 5.0. |
存储 |
Store at ≤-70°C, stable for 6 months after receipt. Store at ≤-70°C, stable for 3 months under sterile conditions after opening. Please minimize freeze-thaw cycles. |
运输方式 |
The product is shipped on dry ice/polar packs. Upon receipt, store it immediately at the temperature listed below. |
研究背景 | Medium-Chain Specific Acyl-CoA Dehydrogenase (ACADM) is a mitochondrial fatty acid beta-oxidation that belongs to the acyl-CoA dehydrogenase family. ACADM is a homotetramer enzyme that catalyzes the initial step of the mitochondrial fatty acid beta-oxidation pathway. ACADM is specific for acyl chain lengths of 4 to 16. It is essential for converting these particular fatty acids to energy, especially during fasting periods. Defects in ACADM cause medium-chain acyl-CoA dehydrogenase deficiency, a disease characterized by hepatic dysfunction, fasting hypoglycemia, and encephalopathy, which can result in infantile death. |
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ACADM Protein, Human, Recombinant (His) ACADM Medium-Chain Specific Acyl-CoA Dehydrogenase Mitochondrial MCAD recombinant recombinant-proteins proteins protein