@article{MTMT:1426941, title = {An enzymatic method for the determination of butyrobetaine via conversion to carnitine after isolation by high performance liquid chromatography.}, url = {https://m2.mtmt.hu/api/publication/1426941}, author = {Sándor, Attila and Minkler, PE and Ingalls, ST and Hoppel, CL}, doi = {10.1016/0009-8981(88)90170-2}, journal-iso = {CLIN CHIM ACTA}, journal = {CLINICA CHIMICA ACTA}, volume = {176}, unique-id = {1426941}, issn = {0009-8981}, abstract = {An improved procedure for the determination of butyrobetaine [4-(N,N,N-trimethylammonio)butanoate] in plasma and tissue is described. Butyrobetaine was isolated by ion-exchange chromatography and high performance liquid chromatography. The isolation procedure was internally standardized with [3H]butyrobetaine. The recovery of butyrobetaine was greater than 90%. Following isolation butyrobetaine was enzymatically converted to carnitine using butyrobetaine hydroxylase and the resulting carnitine was assayed using carnitine acetyltransferase and [14C]acetylcoenzyme A. The conversion of butyrobetaine to carnitine and of carnitine to [14C]acetylcarnitine was greater than 98% as determined by high performance liquid chromatography. Using this method was analysed human sera (healthy controls) and tissues (autopsy) and found the following values: serum, 4.67 nmol/ml; kidney 17.6 nmol/g; liver, 26.5 nmol/g. The serum butyrobetaine values of twins suffering from carnitine deficiency were normal (3.78 and 3.87 nmol/ml), while the carnitine supplementation therapy caused an increase. Animal samples were analyzed and the values were 3-4 times higher than previously reported by others.}, keywords = {Animals; Male; Humans; RATS; Chromatography, High Pressure Liquid; Rats, Inbred Strains; Child, Preschool; Methods; Infant; Infant, Newborn; Carnitine/*metabolism; Betaine/*analogs & derivatives/analysis}, year = {1988}, eissn = {1873-3492}, pages = {17-27} }