L-Palmitoylcarnitine

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L-Palmitoylcarnitine
2D structure for L-Palmitoylcarnitine
Chemical Name	(3R)-3-hexadecanoyloxy-4-trimethylazaniumylbutanoate
Chemical Formula	C₂₃H₄₅NO₄
CAS Number	2364-67-2
Chemical Information	HMDB00222
Biochemical Taxonomy	Carnitines
Functional Taxonomy	Not Available
Nutritional Taxonomy	Not Available
Metabolic Pathways	Fatty Acid Metabolism
Biofluid Location	Blood Urine
Tissue Location	Not Available
Normal Biofluid Concentrations	Blood: 0.113 +/- 0.006 umol/L Urine: 0.5 umol/mmol creatinine
Normal Tissue Concentrations	Not Available
Diseases / Conditions Related to Nutrition	Patients with celiac disease
Other (Monogenic Disorders)	Carnitine palmitoyltransferase II deficiency, late-onset OMIM: 255110
Abnormal Biofluid Concentrations	Blood (Patients with celiac disease): 0.097 +/- 0.006 umol/L
Abnormal Tissue Concentrations	Not Available
Physiological Processes	Not Available

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Introduction

guidelines
L- Palmitoylcarnitine is a long-chain acyl fatty acid derivative ester of carnitine which facilitates the transfer of long-chain fatty acids from cytoplasm into mitochondria during the oxidation of fatty acids. L- palmitoylcarnitine, due to its amphipatic character is, like detergents, a surface-active molecule and by changing the membrane fluidity and surface charge can change activity of several enzymes and transporters localized in the membrane. L-palmitoylcarnitine has been also reported to change the activity of certain proteins. On the contrary to carnitine, palmitoylcarnitine was shown to stimulate the activity of caspases 3, 7 and 8 and the level of this long-chain acylcarnitine increased during apoptosis. Palmitoylcarnitine was also reported to diminish completely binding of phorbol esters, the protein kinase C activators and to decrease the autophosphorylation of the enzyme. Apart from these isoform nonspecific phenomena, palmitoylcarnitine was also shown to be responsible for retardation in cytoplasm of protein kinase C isoforms β and δ and, in the case of the latter one, to decrease its interaction with GAP-43. Some of the physico-chemical properties of palmitoylcarnitine may help to explain the need for coenzyme A-carnitine-coenzyme A acyl exchange during mitochondrial fatty acid import. The amphiphilic character of palmitoylcarnitine may also explain its proposed involvement in the pathogenesis of myocardial ischemia. L-Palmitoylcarnitine accumulates in ischemic myocardium and potentially contribute to myocardial damage through alterations in membrane molecular dynamics , one mechanism through which could play an important role in ischemic injury. Palmitoylcarnitine is characteristically elevated in carnitine palmitoyltransferase II deficiency, late-onset (OMIM 255110). (PMID 2540838, 15363641, 8706815)