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品种反应性 主要应用 宿主 格式 抗体类型 R WB Rb Serum Polyclonal Antibody 描述 产品目录编号 ABS85 描述 Anti-CPT2 Antibody Alternate Names - CPT II
- Carnitine O-palmitoyltransferase 2, mitochondrial
- Carnitine palmitoyltransferase II
背景信息 Carnitine palmitoyltransferase II (CPT2, or CPT II) belongs to the carnitine/choline acetyltransferase family. It is a ubiquitous protein found in the inner membrane of the mitochondria and plays an essential role in fatty acid β-oxidation. CPT2 is part of the system involving CPT1 and carnitine/acylcarnitine translocase (CACT), that facilitate the transport of long chain fatty acids into the mitochondrial matrix. CPT2 plays the role of restoring these molecules to original esterified forms suitable for β-oxidation, after they arrive in the matrix. Defects in CPT2 has varying outcomes: in adults, it results in episodes of rhabdomyolysis (breakdown of muscle fibers which circulate in the bloodstream), after prolonged and vigorous exercise; and in infants, CPT2 deficiency induces hypoketotic hypoglycemia and eventual cardiac damage. 产品信息 格式 Serum 控制 - Rat liver mitochondria tissue lysate
演示 Rabbit polyclonal serum containing 0.05% sodium azide. 应用 应用 Anti-CPT2 detects levels of CPT2 proteins & has been published & validated for use in WB. 主要应用 - Western Blotting
生物信息 免疫原品种 KLH-conjugated linear peptide corresponding to human CPT2. 宿主 Rabbit 同种型 IgG 品种反应性 Rat Species Reactivity Note Demonstrated to react in Rat. Predicted to react with Human based on immunogen design. 抗体类型 Polyclonal Antibody Entrez基因编号 - NP_000089
Entrez基因汇总 The protein encoded by this gene is a nuclear protein which is transported to the mitochondrial inner membrane. Together with carnitine palmitoyltransferase I, the encoded protein oxidizes long-chain fatty acids in the mitochondria. Defects in this gene are associated with mitochondrial long-chain fatty-acid (LCFA) oxidation disorders. [provided by RefSeq, Jul 2008]. 基因符号 - CPT1
- CPT2
纯化方法 Unpurified UniProt编号 - P23786
UniProt汇总 CATALYTIC ACTIVITY: Palmitoyl-CoA + L-carnitine = CoA + L-palmitoylcarnitine.
PATHWAY: Lipid metabolism; fatty acid beta-oxidation.
SUBCELLULAR LOCATION: Mitochondrion inner membrane; Peripheral membrane protein; Matrix side.
INVOLVEMENT IN DISEASE: Defects in CPT2 are the cause of carnitine palmitoyltransferase 2 deficiency late-onset (CPT2D) [MIM:255110]; also known as CPT-II deficiency or CPT2 deficiency. CPT2D is an autosomal recessive disorder characterized by recurrent myoglobinuria, episodes of muscle pain, stiffness, and rhabdomyolysis. These symptoms are triggered by prolonged exercise, fasting or viral infection and patients are usually young adults. In addition to this classical, late-onset, muscular type, a hepatic or hepatocardiomuscular form has been reported in infants. Clinical pictures in these children or neonates include hypoketotic hypoglycemia, liver dysfunction, cardiomyopathy and sudden death. Ref.3 Ref.11 Ref.12 Ref.13 Ref.14 Ref.15 Ref.16 Ref.17 Ref.18 Ref.20 Ref.21 Ref.22
Defects in CPT2 are the cause of carnitine palmitoyltransferase 2 deficiency infantile (CPT2DI) [MIM:600649]. A disorder of mitochondrial long-chain fatty acid oxidation characterized by hepatic or hepato-cardio-muscular manifestations with onset in infancy. Clinical features include hypoketotic hypoglycemia, lethargy, seizures, hepatomegaly, liver dysfunction, cardiomegaly and dilated cardiomyopathy.
Defects in CPT2 are the cause of carnitine palmitoyltransferase 2 deficiency lethal neonatal (CPT2D-LN) [MIM:608836]; also known as lethal neonatal CPT-II deficiency. It is a lethal neonatal form of CPT2D. This rarely presentation is antenatal with cerebral periventricular cysts and cystic dysplastic kidneys. The clinical variability of the disease is likely attributed to the variable residual enzymatic activity.
Defects in CPT2 are a cause of susceptibility to encephalopathy acute infection-induced type 4 (IIAE4) [MIM:614212]. A severe neurologic complication of an infection. It manifests within days in otherwise healthy children after common viral infections, without evidence of viral infection of the brain or inflammatory cell infiltration. In affected children, high-grade fever is accompanied within 12 to 48 hours by febrile convulsions, often leading to coma, multiple-organ failure, brain edema, and high morbidity and mortality. The infections are usually viral, particularly influenza, although other viruses and even mycoplasma have been found to cause the disorder. Note=Polymorphic variants in CPT2 can confer susceptibility to infection-induced encepalopathy. These variants do not cause classical carnitine palmitoyltransferase 2 deficiency, and patients harboring any of them are asymptomatic most of the time. However, they are prone to viral infection (high fever)-related encephalopathy (Ref.25). Ref.23 Ref.25
SEQUENCE SIMILARITIES: Belongs to the carnitine/choline acetyltransferase family.产品使用声明 质量保证 Evaluated by Western Blot in rat liver mitochondria tissue lysate.
Western Blot Analysis: A 1:1,000 dilution of this antibody detected CPT2 on 10 µg of rat liver mitochondria 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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