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品种反应性 主要应用 宿主 格式 抗体类型 M, R WB Rb Serum Polyclonal Antibody 描述 产品目录编号 ABS65 描述 Anti-CPT1A Antibody Alternate Names - Carnitine palmitoyltransferase 1A
- Carnitine O-palmitoyltransferase 1, liver isoform
- CPT1-L
- Carnitine O-palmitoyltransferase I, liver isoform
- CPT I
- CPTI-L
背景信息 Carnitine palmitoyltransferase 1A (CPT1A, CPT1-L, or L-CPT1) is an hepatic enzyme that, together with CPT2 and carnitine-acylcarnitine translocase, mediates the oxidation of long chain fatty acids to produce energy, in periods of fasting. CPT1A is localized in the outer mitochondrial membrane and catalyzes the addition of carnitine to long-chain fatty acids thereby enabling effective transport of these molecules to the inner mitochondrial membrane where they are metabolized. CPT1A may function as part of a complex consisting of the acyl-CoA synthetase (ACSL) and the voltage-dependent anion channel (VDAC) and is regulated by malonyl-CoA. Deficiencies in CPT1A result in hypoketotic hypoglycemia. 产品信息 格式 Serum 控制 - Mouse liver tissue lysate
演示 Rabbit polyclonal serum containing 0.05% sodium azide. 应用 应用 Detect CPT1A using this Anti-CPT1A Antibody validated for use in Western Blotting. 主要应用 - Western Blotting
生物信息 免疫原品种 GST-tagged recombinant protein corresponding to rat CPT1A. 宿主 Rabbit 同种型 IgG 品种反应性 MouseRat Species Reactivity Note Demonstrated to react with Rat and Mouse. 抗体类型 Polyclonal Antibody Entrez基因编号 - NP_001867
Entrez基因汇总 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]. 基因符号 - CPT1
- CPT1A
纯化方法 Unpurified UniProt编号 - P50416
UniProt汇总 CATALYTIC ACTIVITY: Palmitoyl-CoA + L-carnitine = CoA + L-palmitoylcarnitine.
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.
SUBCELLULAR LOCATION: Mitochondrion outer membrane; Multi-pass membrane protein.
TISSUE SPECIFICITY: Strong expression in kidney and heart, and lower in liver and skeletal muscle.
INVOLVEMENT IN DISEASE: Defects in CPT1A are the cause of carnitine palmitoyltransferase 1A deficiency (CPT1AD) [MIM:255120]; also known as CPT-I deficiency or CPT1A deficiency. CPT1AD 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.
SEQUENCE SIMILARITIESl Belongs to the carnitine/choline acetyltransferase family.产品使用声明 质量保证 Evaluated by Western Blot in mouse liver tissue lysate.
Western Blot Analysis: A 1:1,000 dilution of this antibody detected CPT1A on 10 µg of mouse liver tissue lysate.使用声明 - Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
储存和货运信息 存储条件 Stable for 1 year at -20°C from date of receipt.
Handling Recommendations: Upon receipt and prior to removing the cap, centrifuge the vial and gently mix the solution. Aliquot into microcentrifuge tubes and store at -20°C. Avoid repeated freeze/thaw cycles, which may damage IgG and affect product performance.包装信息 数量 100 µL
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