Alexa Fluor® 647 Anti-CPT2 antibody [EPR13626] - C-terminal

Supplementary info

This supplementary information is collated from multiple sources and compiled automatically.

Activity summary

Carnitine palmitoyltransferase 2 (CPT2) also known as CPT-2 is an enzyme located mainly in the mitochondria of liver muscle and heart tissues. This enzyme has a molecular mass of approximately 68 kDa. CPT2 plays a mechanical role in the transfer of long-chain fatty acids from the cytosol into the mitochondrial matrix completing the process initiated by CPT1 on the outer mitochondrial membrane. CPT2 acts on the inner mitochondrial membrane converting acylcarnitine back into acyl-CoA which is an essential step in beta-oxidation. Expression of CPT2 is higher in tissues with elevated fatty acid oxidation rates.

Biological function summary

CPT2 is integral to the mitochondrial pathway of fatty acid metabolism. It functions as part of the carnitine shuttle system a complex important for the conversion of acylcarnitine to acyl-CoA inside the mitochondria. This role is vital for the full oxidation of long-chain fatty acids. Through its action CPT2 aids in maintaining energy homeostasis and producing ketone bodies especially during periods of fasting or strenuous exercise.

Pathways

CPT2 is involved in both the beta-oxidation and the carnitine shuttle pathways. These pathways facilitate the energy production from fatty acids particularly during states of low carbohydrate availability. CPT2 works closely with CPT1 as both enzymes form the gateway for the entry of long-chain fatty acids into mitochondria. Together with enzymes from the tricarboxylic acid cycle CPT2 helps sustain ATP production under such metabolic conditions.

Associated diseases and disorders

Defects in CPT2 can lead to disorders like CPT2 deficiency which present with symptoms of muscle weakness and recurrent rhabdomyolysis especially during prolonged fasting or significant exercise. This deficiency can also result in hypoketotic hypoglycemia and liver dysfunction. CPT2-related disorders often involve altered interactions with proteins such as the acyl-CoA dehydrogenases impacting the normal fatty acid oxidation process and causing metabolic disturbances.