Typical Testing Data/Standard Curve (for reference only)
Guinea Pig Carnitine O-palmitoyltransferase 2, mitochondrial (CPT2) ELISA Kit | CPT2 elisa kit
Guinea pig Carnitine O-palmitoyltransferase 2, mitochondrial (CPT2) ELISA Kit
Typical Testing Data/Standard Curve (for reference only)
Typical Testing Data/Standard Curve (for reference only)
Principle of the Assay: CPT2 ELISA kit applies the competitive enzyme immunoassay technique utilizing an anti-CPT2 antibody and an CPT2-HRP conjugate. The assay sample and buffer are incubated together with CPT2-HRP conjugate in pre-coated plate for one hour. After the incubation period, the wells are decanted and washed five times. The wells are then incubated with a substrate for HRP enzyme. The product of the enzyme-substrate reaction forms a blue colored complex. Finally, a stop solution is added to stop the reaction, which will then turn the solution yellow. The intensity of color is measured spectrophotometrically at 450nm in a microplate reader. The intensity of the color is inversely proportional to the CPT2 concentration since CPT2 from samples and CPT2-HRP conjugate compete for the anti-CPT2 antibody binding site. Since the number of sites is limited, as more sites are occupied by CPT2 from the sample, fewer sites are left to bind CPT2-HRP conjugate. A standard curve is plotted relating the intensity of the color (O.D.) to the concentration of standards. The CPT2 concentration in each sample is interpolated from this standard curve.
NCBI and Uniprot Product Information
NCBI Description
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]
Uniprot Description
CPT2: Defects in CPT2 are the cause of carnitine palmitoyltransferase 2 deficiency late-onset (CPT2D); 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. Defects in CPT2 are the cause of carnitine palmitoyltransferase 2 deficiency infantile (CPT2DI). 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); 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). 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. 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 (PubMed:21697855). Belongs to the carnitine/choline acetyltransferase family.
Protein type: Mitochondrial; Lipid Metabolism - fatty acid; EC 2.3.1.21; Transferase
Chromosomal Location of Human Ortholog: 1p32
Cellular Component: nucleoplasm; mitochondrion; mitochondrial inner membrane; nucleolus
Molecular Function: carnitine O-palmitoyltransferase activity
Biological Process: fatty acid beta-oxidation; cellular lipid metabolic process; carnitine shuttle
Disease: Carnitine Palmitoyltransferase Ii Deficiency, Lethal Neonatal; Carnitine Palmitoyltransferase Ii Deficiency, Infantile; Encephalopathy, Acute, Infection-induced, Susceptibility To, 4; Carnitine Palmitoyltransferase Ii Deficiency, Late-onset
Research Articles on CPT2
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Product Notes
The Guinea Pig CPT2 cpt2 (Catalog #AAA7240748) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA7240748 ELISA Kit recognizes Guinea Pig CPT2. It is sometimes possible for the material contained within the vial of "Carnitine O-palmitoyltransferase 2, mitochondrial (CPT2), ELISA Kit" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
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