Carnitine Palmitoyl Transferase 2 (CPT-2) | MyBioSource MBS612629 product information

This webpage contains legacy information. The product is either no longer available from the supplier or has been delisted at Labome.

product summary

company name :

MyBioSource

product type :

antibody

product name :

Carnitine Palmitoyl Transferase 2 (CPT-2)

catalog :

MBS612629

quantity :

0.05 mg

price :

400 USD

clonality :

polyclonal

host :

rabbit

conjugate :

nonconjugated

reactivity :

human, mouse

application :

western blot, ELISA, enzyme immunoassay

product information

catalog number :

MBS612629

products type :

Antibody

products full name :

Carnitine Palmitoyl Transferase 2 (CPT-2)

products short name :

Carnitine Palmitoyl Transferase 2

products name syn :

Anti -Carnitine Palmitoyl Transferase 2 (CPT-2)

other names :

carnitine O-palmitoyltransferase 2, mitochondrial; Carnitine O-palmitoyltransferase 2, mitochondrial; carnitine O-palmitoyltransferase 2, mitochondrial; CPT II; OTTHUMP00000010493; carnitine palmitoyltransferase II; carnitine palmitoyltransferase 2; Carnitine palmitoyltransferase II

other gene names :

CPT2; CPT2; CPT1; CPTASE; CPT1

uniprot entry name :

CPT2_HUMAN

clonality :

Polyclonal

isotype :

IgG

host :

Rabbit

reactivity :

Mouse

specificity :

Recongizes mouse CPT-2

purity :

Affinity Purified. Purified by immunoaffinity chromatography.

form :

Supplied as a liquid in PBS, pH 7.4 and 0.1% sodium azide, 40% glycerol.

storage stability :

May be stored at 4 degree C for short-term only. For long-term storage, aliquot and store at -20 degree C. Aliquots are stable for at least 12 months at -20 degree C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.

tested application :

ELISA (EL/EIA), Western Blot (WB)

app notes :

Suitable for use in ELISA, Western Blot. Dilution: Western Blot: 1-10ug/ml. ELISA: 1:10,000-1:100,000 using 50-100ng of control peptide/well. Optimal dilutions to be determined by the researcher.

other info1 :

Immunogen: 16-aa peptide sequence mapping near the C-terminus of mouse CPT-2L (KLH coupled). Species Sequence Homology: rat: 93%; human: 86%. No significant sequence homology is seen with CPT-1 or any other protein.

products categories :

Antibodies; Abs to Enzymes

products description :

In fatty acid synthesis, catalytic formation of malonyl-CoA (precursor for long-chain fatty acyl-CoA, LCFA-CoA) from acetyl-CoA by Acetyl-CoA carboxylase (ACC-1) is the rate limiting step. The translocation of LCFA-CoA from cytosol to mitochondria is catalyzed by two carnitine palmitoyl transferases (CPT-1 & CPT-2) and regulated by ACC-2, the rate limiting step of mitochondrial fatty acid b-oxidation. Activities of ACC-1 and 2 are regulated by their phosphorylation by 5'-AMP-activated protein kinase (AMPK). Diabetes deranges AMPK master-switch and represses the ACC-1 gene-expression and stimulates excessive fatty acid oxidation which in turn interferes with glucose metabolism. Mitochondrial oxidation of LC-FCA is initiated by the sequential action of CPT-1, which is located in the outer membrane, and CPT-2, which is located in the inner membrane together with a carnitine-acylcarnitine translocase. CPT-2 (mouse/rat/human 658-aa, ~74kD, chromosome 1p32, ~20% identity with CPT1) is a ubiquitous malonyl-CoA-insensitive transferase localized in the inner mitochondrial membrane. It catalyzes the re-synthesis of acyl-CoA from acyl-carnitines. CPT-2 deficiency leads to the most commonly inherited, lipid myopathy in adults characterized by exercise-induced pain, stiffness, and myoglobinuria. The aa sequences of the two isoforms are ~61% identical.

ncbi gi num :

4503023

ncbi acc num :

NP_000089.1

ncbi gb acc num :

NM_000098.2

uniprot acc num :

P23786

ncbi mol weight :

73,777 Da

ncbi pathways :

Fatty Acid Beta Oxidation Pathway (198865); Fatty Acid Metabolism Pathway (82935); Fatty Acid Metabolism Pathway (296); Fatty Acid, Triacylglycerol, And Ketone Body Metabolism Pathway (160977); Import Of Palmitoyl-CoA Into The Mitochondrial Matrix Pathway (106108); Metabolism Of Lipids And Lipoproteins Pathway (160976); Mitochondrial LC-Fatty Acid Beta-Oxidation Pathway (198817); PPAR Signaling Pathway (83042); PPAR Signaling Pathway (450); Regulation Of Lipid Metabolism By Peroxisome Proliferator-activated Receptor Alpha (PPARalpha) Pathway (119544)

ncbi summary :

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]

uniprot summary :

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; Transferase; EC 2.3.1.21. 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

size1 :

0.05 mg

price1 :

400 USD