Quantification of free carnitine, individual short- and medium-chain acylcarnitines, and total carnitine in plasma by high-performance liquid chromatography.
This paper describes a method for the quantitative determination of free carnitine, acetylcarnitine, propionylcarnitine, hexanoylcarnitine, octanoylcarnitine, and total carnitine in plasma. Carnitine and acylcarnitines were extracted from 100 microliters of plasma with acetonitrile/methanol and isolated using 0.5-ml columns of silica gel. Samples were then derivatized with 4'-bromophenacyl trifluoromethanesulfonate and quantified by high-performance liquid chromatography with detection at 260 nm. Carnitine and acylcarnitines were quantified in normal human plasma and the plasma of patients diagnosed with methylmalonic aciduria, propionic acidemia, and medium-chain acyl-CoA dehydrogenase deficiency.
Minkler
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PE
Medical Research Service, Department of Veterans Affairs Medical Center, Cleveland, Ohio 44106.
Hoppel
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D000108:Acetylcarnitine / Q000097:blood; D042964:Acyl-CoA Dehydrogenase; D044944:Acyl-CoA Dehydrogenases / Q000172:deficiency; D000215:Acylation; D002331:Carnitine / Q000031:analogs & derivatives* / Q000097:blood*; D002851:Chromatography, High Pressure Liquid; D006801:Humans; D008764:Methylmalonic Acid / Q000652:urine; D008970:Molecular Weight; D011422:Propionates / Q000097:blood; D012015:Reference Standards; D015203:Reproducibility of Results
D011422:Propionates; C061301:hexanoylcarnitine; C003223:propionylcarnitine; D000108:Acetylcarnitine; D008764:Methylmalonic Acid; D044944:Acyl-CoA Dehydrogenases; D042964:Acyl-CoA Dehydrogenase; C029658:propionic acid; C008698:octanoylcarnitine; D002331:Carnitine