描述
Vaccenyl carnitine is a long-chain acyl fatty acid derivative ester of carnitine. Long-chain acyl fatty acid derivatives accumulate in the cytosol and serum of patients suffering of mitochondrial carnitine palmitoyltransferase II (CPT II, EC 2.3.1.12) deficiency, the most common inherited disorder of lipid metabolism in adults. carnitine palmitoyltransferase II deficiency is an autosomal recessive disorder of fatty acid metabolism presenting as two clinical phenotypes: (i) a severe infantile hepatocardiomuscular form and (ii) a milder adult muscular form. Energy production from long-chain fatty acids (LCFAs) requires the transport of LCFAs into the mitochondrial matrix. This transport is carnitine-dependent and involves translocation machinery. mitochondrial fatty acid oxidation disorders cause hypoglycaemia, hepatic dysfunction, myopathy, cardiomyopathy and encephalopathy. Patients with end-stage renal disease (ESRD) undergoing long-term haemodialysis exhibit elevated acylcarnitine concentrations. (PMID: 11999976, 10682306, 15025677, 16168195) [HMDB]
InChI标识符
InChI=1S/C25H49NO3/c1-5-6-7-8-9-10-11-12-13-14-15-16-17-18-19-20-21-29-24(22-25(27)28)23-26(2,3)4/h10-11,24H,5-9,12-23H2,1-4H3