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Unit Code 88830:
C5-OH Acylcarnitine, Quantitative, Urine

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Method Description

Acylcarnitines, including 3-hydroxy isovalerylcarnitine, 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

Key