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.
パッケージサイズ | 在庫状況 | 単価(税別) |
---|---|---|
10 μg | 約5 days | ¥ 30,000 |
50 μg | 約5 days | ¥ 89,500 |
500 μg | 約5 days | ¥ 436,000 |
1 mg | 約5 days | ¥ 626,500 |
説明 | 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. |
Species | Human |
Expression Host | E. coli |
Tag | N-6xHis |
Accession Number | P11310 |
別名 | ACADM, Medium-Chain Specific Acyl-CoA Dehydrogenase Mitochondrial, MCAD |
Amino Acid | Lys26-Asn421 |
Construction | Lys26-Asn421 |
Protein Purity | Greater than 95% as determined by reducing SDS-PAGE. (QC verified) |
分子量 | 42 KDa (reducing condition) |
Endotoxin | < 1 EU/μg by the LAL method. |
Formulation | Supplied as a 0.2 μm filtered solution of 20 mM Acetate, 10% Trehalose, 0.05% Tween 80, pH 5.0. |
Stability & Storage |
Lyophilized powders can be stably stored for over 12 months, while liquid products can be stored for 6-12 months at-80℃. For reconstituted proteinsolutions, the solution can be stored at -20°c to -80'c for at least 3 months. Please avoid multiple freeze-thaw cycles and store products in aliquots. |
Shipping |
In general, Lyophilized powders are shipping with blue ice. Solutions are shipping with dry ice. |
Research Background | 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