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| Values are valid only on day of printing. | |
Acylcarnitines, including iso-butyrylcarnitine, are determined in
urine by flow injection analysis tandem mass spectrometry
using acetyl-d(3)-carnitine, propionyl-d(3)-carnitine,
butyryl-d(3)-carnitine, octanoyl-d(3)-carnitine, dodecanoyl-d(3)-carnitine,
and palmitoyl-d(3)-carnitine as internal standards. A total of 20 uL
of the diluted urine is extracted with an acidified acetonitrile
solution containing the internal standards. The supernatant is
evaporated and the residue treated with n-butanolic HCl yielding
the acylcarnitines for analysis as their n-butyl esters. (Tortorelli S,
Hahn SH, Cowan TM, et al: The urinary excretion of glutarylcarnitine
is an informative tool in the biochemical diagnosis of glutaric
acidemia type I. Mol Genet Metab 2005;84:137-143)

