Unit Code 88831:
C5-DC Acylcarnitine, Quantitative, Urine
Method Description
Acylcarnitines, including glutarylcarnitine, 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)
Performing Laboratory Location
Rochester
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