Carnitine O-palmitoyltransferase
Carnitine acyltransferase system is an important biological transport system comprising a plurality of enzymes, which is used for the transport of fatty acids into the mitochondria. There is derived from the fatty acids in the beta- oxidation energy.
Basics
Triglycerides consisting of fatty acids and glycerol and are important energy storage devices. You can take the food intake via the esophagus and stomach into the small intestine and are cleaved by lipolysis using the pancreatic lipase into free fatty acids and glycerol. The two products of lipolysis, can be used as complex with bile acids, so-called micelles absorbed, that is to be taken up by the epithelial cells of the small intestine. In the cells, triglycerides are formed from the free fatty acids and glycerol again, packed with apolipoproteins and are transported in the form of chylomicrons through the lymphatic system and bloodstream to the tissues.
The transport of fatty acids from the cytosol into the mitochondrial matrix, the location of the beta- oxidation is catalyzed by various carnitine acyltransferases, acyl -CoA synthetase, and the carnitine / acylcarnitine translocase. According to current understanding, there are three different carnitine acyltransferases with different specificities for different chain lengths of the substrates. Specifically, these are the carnitine acyltransferase (CAT ), the carnitine Oktanyltransferase (COT ) and carnitine Palmityltransferase (CPT ). In contrast to the CPT Group, CAT and COT are completely soluble.
Genetics
The carnitine Palmityltransferasesystem consists of two immunologically identifiable against each encoded on different genes components: the carnitine Palmityltransferase 1 (CPT -1, EC 2.3.1.21 ) and the carnitine Palmityltransferase 2 (CPT -2). While the CPT 2 as a single protein associated with the locus 1p32, exist for the isoforms of CPT -1, each with its own genes, so that the L- 1 CPT on chromosome 11q13 and Muskelisoform (M -CPT 1) encoded on chromosome 22q13.3 is.