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Quantitation of Long-Chain Acylcarnitines by HPLC/fluorescence Detection: Application to Plasma and Tissue Specimens From Patients With Carnitine palmitoyltransferase-II Deficiency

Quantitation of Long-Chain Acylcarnitines by HPLC/fluorescence Detection: Application to Plasma and Tissue Specimens From Patients With Carnitine palmitoyltransferase-II Deficiency

Paul E Minkler, Janos Kerner, Kathryn N North, Charles L Hoppel

Clin Chim Acta. 2005 Feb;352(1-2):81-92.

PMID: 15653102

Abstract:

Background:
Carnitine palmitoyltransferase-II deficiency (CPT-II deficiency) is a rare disorder of lipid metabolism, in which the accumulation of long-chain acylcarnitines is a diagnostic marker. HPLC with fluorescence detection is an attractive analysis method due to its favorable combination of sensitivity, specificity, ease of analysis and minimal capital equipment costs.
Methods:
Long-chain acylcarnitines were isolated from tissue homogenates (0.5-2 mg wet weight) or plasma (50 microl) using silica gel columns and derivatized with 2-(2,3-naphthalimino)ethyl trifluoromethanesulfonate. Quantitation was by HPLC and fluorescence detection with standard curves (0.0-5.0 nmol/ml) for myristoyl-, palmitoleoyl-, palmitoyl-, oleoyl- and stearoylcarnitine using heptadecanoylcarnitine as the internal standard.
Results:
Significantly greater amounts of long-chain acylcarnitines were quantified in patients with CPT-II deficiency when compared to controls; e.g. (nmol/ml in patient plasma, controls mean+/-standard deviation): myristoylcarnitine (0.3, not detectable), palmitoleoylcarnitine (0.5, 0.1+/-0.1), palmitoylcarnitine (0.9, 0.1+/-0.0), oleoylcarnitine (3.0, 0.2+/-0.1), stearoylcarnitine (0.4, not detectable).
Conclusions:
This method can be used to quantitate long-chain acylcarnitines, illustrating their accumulation in CPT-II deficiency. The analysis was accomplished using inexpensive and widely available instrumentation and is appropriate for research investigators who require precise quantitation of long-chain acylcarnitines in complex biological samples.

Chemicals Related in the Paper:

Catalog Number	Product Name	Structure	CAS Number	Price
AP425752	Ethyl trifluoromethanesulfonate		425-75-2	Price

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