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Unit Code 88829:
C4 Acylcarnitine, Quantitative, Urine

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Method Description

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)

Performing Laboratory Location

Rochester

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