CPT1A
GeneName
CPT1A
Summary
CPT1A, also known as CPT1, CPT-1, or CPT I, is an 88 kDa enzyme located in the mitochondrial outer membrane. It plays a critical role in the carnitine shuttle, facilitating the transport of long-chain fatty acids into the mitochondria for beta-oxidation. CPT1A exhibits carnitine O-palmitoyltransferase activity, enabling the conversion of acyl-CoA to acylcarnitine, which is essential for fatty acid metabolism. This protein is involved in various biological processes, including the regulation of insulin secretion, liver regeneration, and responses to nutritional changes. Its expression is particularly significant in tissues with high fatty acid oxidation demands, such as the liver and muscle.
Importance
CPT1A is relevant to: - Metabolic disorders, including obesity and type 2 diabetes, due to its role in fatty acid metabolism and insulin regulation. - Liver function and regeneration, as it is involved in lipid metabolism and energy homeostasis. - The understanding of carnitine metabolism and its implications in conditions like carnitine deficiency. - The regulation of innate immune responses, linking metabolic processes with immune function.
Top Products
For researchers investigating CPT1A, we recommend two excellent primary antibodies. The first is the well-cited Anti-CPT1A antibody [8F6AE9] (ab128568), which has garnered 224 citations, reflecting its strong reputation in the field. This monoclonal antibody is validated for use in various applications, including Western blotting (WB), immunohistochemistry (IHC), immunocytochemistry (ICC), flow cytometry (FC), and ELISA, making it a versatile choice for your research needs. Importantly, it has also been validated in knockout models, ensuring reliable performance.Additionally, we offer the recombinant antibody Anti-CPT1A antibody [EPR21843-71-2F] (ab234111). This product is also validated in knockout models and is suitable for WB, IHC, and FC applications. With 57 citations, it demonstrates a solid presence in the research community. The recombinant nature of this antibody provides batch-to-batch consistency, making it an excellent option for researchers seeking reliable CPT1A detection.
Abcam Product Citation Summary
The data indicates that CPT1A is primarily studied in the context of lipid metabolism, particularly in mouse liver tissues. Additionally, there are studies involving human HepG2 cells that explore the regulation of CPT1A by LRRK2. The use of CPT1A antibodies in various experimental contexts highlights its significance in metabolic studies and potential implications in heart failure and chronic diseases.
Abcam Product Citation Table
Domain
A conformation change in the N-terminal region spanning the first 42 residues plays an important role in the regulation of enzyme activity by malonyl-CoA.
Function
Catalyzes the transfer of the acyl group of long-chain fatty acid-CoA conjugates onto carnitine, an essential step for the mitochondrial uptake of long-chain fatty acids and their subsequent beta-oxidation in the mitochondrion (PubMed:11350182, PubMed:14517221, PubMed:16651524, PubMed:9691089). Possesses also a lysine succinyltransferase activity that can regulate enzymatic activity of substrate proteins such as ENO1 and metabolism independent of its classical carnitine O-palmitoyltransferase activity (PubMed:29425493). Plays an important role in hepatic triglyceride metabolism (By similarity). Plays also a role in inducible regulatory T-cell (iTreg) differentiation once activated by butyryl-CoA that antagonizes malonyl-CoA-mediated CPT1A repression (By similarity). Sustains the IFN-I response by recruiting ZDHCC4 to palmitoylate MAVS at the mitochondria leading to MAVS stabilization and activation (PubMed:38016475). Promotes ROS-induced oxidative stress in liver injury via modulation of NFE2L2 and NLRP3-mediated signaling pathways (By similarity).
Involvement in disease
Carnitine palmitoyltransferase 1A deficiency
CPT1AD
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.
None
The disease is caused by variants affecting the gene represented in this entry.
Pathway
Lipid metabolism; fatty acid beta-oxidation.
Sequence Similarities
Belongs to the carnitine/choline acetyltransferase family.
Tissue Specificity
Strong expression in kidney and heart, and lower in liver and skeletal muscle.
Cellular localization
- Mitochondrion outer membrane
- Multi-pass membrane protein
Alternative names
CPT1, CPT1A, CPT1-L, Carnitine palmitoyltransferase 1A, Succinyltransferase CPT1A, CPT I, CPTI-L