Sign in or Register   Sign in or Register
  |  

Mouse Anti-CPT2 Recombinant Antibody (1C2AE6) (CBMAB-C3725-LY)

This product is antibody recognizes CPT2. The antibody 1C2AE6 immunoassay techniques such as: ELISA, ICC, IP.
See all CPT2 antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
1C2AE6
Antibody Isotype
IgG3
Application
ELISA, ICC, IP

Basic Information

Specificity
Human
Antibody Isotype
IgG3
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Preservative
0.02% sodium azide
Purity
> 95% Purity determined by SDS-PAGE.
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
carnitine palmitoyltransferase II
Introduction
CPT2 (Carnitine Palmitoyltransferase 2) is a Protein Coding gene. Diseases associated with CPT2 include Carnitine Palmitoyltransferase Ii Deficiency, Infantile and Carnitine Palmitoyltransferase Ii Deficiency, Myopathic, Stress-Induced. Among its related pathways are Metabolism and Regulation of lipid metabolism by Peroxisome proliferator-activated receptor alpha (PPARalpha).
Gene Ontology (GO) annotations related to this gene include transferase activity, transferring acyl groups and carnitine O-palmitoyltransferase activity.
Entrez Gene ID
1376
UniProt ID
P23786
Alternative Names
Carnitine Palmitoyltransferase 2; Carnitine Palmitoyltransferase II; EC 2.3.1.21; CPT II; CPT1; Carnitine O-Palmitoyltransferase 2, Mitochondrial; Testicular Secretory Protein Li 13; CPTASE; IIAE4;
Function
Involved in the intramitochondrial synthesis of acylcarnitines from accumulated acyl-CoA metabolites (PubMed:20538056, PubMed:24780397).

Reconverts acylcarnitines back into the respective acyl-CoA esters that can then undergo beta-oxidation, an essential step for the mitochondrial uptake of long-chain fatty acids and their subsequent beta-oxidation in the mitochondrion. Active with medium (C8-C12) and long-chain (C14-C18) acyl-CoA esters (PubMed:20538056).
Biological Process
Carnitine metabolic process Source: UniProtKB
Carnitine shuttle Source: Reactome
Fatty acid beta-oxidation Source: UniProtKB
Long-chain fatty acid metabolic process Source: UniProtKB
Positive regulation of cold-induced thermogenesis Source: YuBioLab
Regulation of metabolic process Source: Reactome
Cellular Location
Mitochondrion inner membrane
Involvement in disease
Carnitine palmitoyltransferase 2 deficiency, myopathic, stress-induced (CPT2D):
An autosomal recessive disorder of mitochondrial long-chain fatty acid oxidation, characterized by recurrent myoglobinuria, episodes of muscle pain, stiffness, and rhabdomyolysis. These symptoms are exacerbated by prolonged exercise, fasting, cold, or viral infection. CPT2DM affects most frequently children or young adults, and severity of attacks is highly variable. Myoglobinuria can cause kidney failure and death.
Carnitine palmitoyltransferase 2 deficiency, infantile (CPT2DI):
An autosomal recessive 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.
Carnitine palmitoyltransferase 2 deficiency, lethal neonatal (CPT2DLN):
An autosomal recessive disorder of mitochondrial long-chain fatty acid oxidation with fatal outcome, presenting shortly after birth. It is characterized by respiratory distress, seizures, altered mental status, hepatomegaly, cardiomegaly, cardiac arrhythmia, and, in many cases, dysmorphic features, renal dysgenesis, and migration defects. recessive.
Encephalopathy, acute, infection-induced, 4 (IIAE4):
Disease susceptibility is associated with variants affecting the gene represented in this entry. CPT2 polymorphic 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). 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.
Topology
Mitochondrial matrix: 26-178
Note=Mitochondrial inner membrane: 179-208
Mitochondrial matrix: 209-658

Joshi, P. R., & Zierz, S. (2020). Muscle carnitine palmitoyltransferase II (CPT II) deficiency: a conceptual approach. Molecules, 25(8), 1784.

Lee, J., Hyon, J. Y., Min, J. Y., Huh, Y. H., Kim, H. J., Lee, H., ... & Han, E. H. (2020). Mitochondrial carnitine palmitoyltransferase 2 is involved in Nε-(carboxymethyl)-lysine-mediated diabetic nephropathy. Pharmacological research, 152, 104600.

Blackburn, M. L., Ono-Moore, K. D., Sobhi, H. F., & Adams, S. H. (2020). Carnitine palmitoyltransferase 2 knockout potentiates palmitate-induced insulin resistance in C2C12 myotubes. American Journal of Physiology-Endocrinology and Metabolism, 319(2), E265-E275.

Pereyra, A. S., Rajan, A., Ferreira, C. R., & Ellis, J. M. (2020). Loss of muscle carnitine palmitoyltransferase 2 prevents diet-induced obesity and insulin resistance despite long-chain acylcarnitine accumulation. Cell reports, 33(6), 108374.

Lu, X., Zhang, X., Zhang, Y., Zhang, K., Zhan, C., Shi, X., ... & Li, Y. (2019). Metabolic profiling analysis upon acylcarnitines in tissues of hepatocellular carcinoma revealed the inhibited carnitine shuttle system caused by the downregulated carnitine palmitoyltransferase 2. Molecular carcinogenesis, 58(5), 749-759.

Shibata, A., Kasai, M., Hoshino, A., Miyagawa, T., Matsumoto, H., Yamanaka, G., ... & Mizuguchi, M. (2019). Thermolabile polymorphism of carnitine palmitoyltransferase 2: a genetic risk factor of overall acute encephalopathy. Brain and Development, 41(10), 862-869.

Tucci, S., Behringer, S., Sturm, M., Grünert, S. C., & Spiekerkoetter, U. (2019). Implementation of a fast method for the measurement of carnitine palmitoyltransferase 2 activity in lymphocytes by tandem mass spectrometry as confirmation for newborn screening. Journal of Inherited Metabolic Disease, 42(5), 850-856.

Wieser, T. (2019). Carnitine palmitoyltransferase II deficiency.

Aires, V., Delmas, D., Djouadi, F., Bastin, J., Cherkaoui-Malki, M., & Latruffe, N. (2018). Resveratrol-induced changes in MicroRNA expression in primary human fibroblasts harboring carnitine-palmitoyl transferase-2 gene mutation, leading to fatty acid oxidation deficiency. Molecules, 23(1), 7.

Pereyra, A. S., Hasek, L. Y., Harris, K. L., Berman, A. G., Damen, F. W., Goergen, C. J., & Ellis, J. M. (2017). Loss of cardiac carnitine palmitoyltransferase 2 results in rapamycin-resistant, acetylation-independent hypertrophy. Journal of Biological Chemistry, 292(45), 18443-18456.

Ask a question We look forward to hearing from you.
0 reviews or Q&As

Purchasing FAQs
Technical FAQs

Loading...
Have you used Mouse Anti-CPT2 Recombinant Antibody (1C2AE6)?
Submit a review and get a Coupon or an Amazon gift card. 20% off Coupon $30 eGift Card
Submit a review
Loading...
For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

Online Inquiry

Documents

Contact us

  • Tel: (USA)
  • (UK)
  • Fax:
  • Email:

Submit A Review

Go to
Compare