| 别名 | Carnitine O-palmitoyltransferase 1, liver isoform;CPT1-L;Carnitine O-palmitoyltransferase I, liver isoform;CPT I;CPTI-L;Carnitine palmitoyltransferase 1A; |
| 基因名称 | CPT1A |
| 蛋白名称 | CPT1A |
| 推荐应用 | |
| 反应物种 | Human,Mouse,Rat |
| 存储缓冲液 | PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA |
| Human Gene Link | 1374 |
| Human Swissprot No. | P50416 |
| Mouse Gene ID | 12894 |
| Mouse Swissprot No. | P97742 |
| Rat Gene ID | 25757 |
| Rat Swissprot No. | P32198 |
| 特异性 | endogenous |
| 稀释度 | WB 1:1000-1:5000；ELISA 1:5000-1:20000 |
| 参考分子量 | MW(Observed):88kD |
| 预测分子量 | MW(Calculated):88kD |
| 运输及保存条件 | -15°C to -25°C/1 year(Do not lower than -25°C) |
| 宿主 | Rabbit |
| 同种型 | IgG,Kappa |
| 背景介绍 | The mitochondrial oxidation of long-chain fatty acids is initiated by the sequential action of carnitine palmitoyltransferase I (which is located in the outer membrane and is detergent-labile) and carnitine palmitoyltransferase II (which is located in the inner membrane and is detergent-stable), together with a carnitine-acylcarnitine translocase. CPT I is the key enzyme in the carnitine-dependent transport across the mitochondrial inner membrane and its deficiency results in a decreased rate of fatty acid beta-oxidation. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008], |
| 细胞定位 | Mitochondrion outer membrane |
| 功能 | Catalytic activity:Palmitoyl-CoA + L-carnitine = CoA + L-palmitoylcarnitine.,Disease:Defects in CPT1A are the cause of carnitine palmitoyltransferase I deficiency (CPT-I deficiency) [MIM:255120]; also known as CPT1A deficiency. CPT I deficiency is a rare autosomal recessive metabolic disorder of long-chain fatty acid oxidation characterized by severe episodes of hypoketotic hypoglycemia usually occurring after fasting or illness. Onset is in infancy or early childhood.,enzyme regulation:Inhibitors such as malonyl-CoA interact with its catalytic domain and not with an associated regulatory component.,pathway:Lipid metabolism; fatty acid beta-oxidation.,similarity:Belongs to the carnitine/choline acetyltransferase family.,tissue specificity:Strong expression in kidney and heart, and lower in liver and skeletal muscle., |
| 纯化 | Protein A |
| Clonality | Monoclonal |
| 修饰类型 | Unmodified |
| 细胞定位 | Mitochondrion outer membrane |
| 组织表达 | Strong expression in kidney and heart, and lower in liver and skeletal muscle. |
| 研究领域 | >>Fatty acid degradation;>>Fatty acid metabolism;>>PPAR signaling pathway;>>AMPK signaling pathway;>>Thermogenesis;>>Adipocytokine signaling pathway;>>Glucagon signaling pathway;>>Insulin resistance;>>Alcoholic liver disease |
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