A new, simple assay for long-chain acyl-CoA dehydrogenase in cultured skin fibroblasts using stable isotopes and GC-MS

K. E. Niezen-Koning; R. J. Wanders; G. T. Nagel; L. IJlst; H. S. Heymans

doi:https://doi.org/10.1016/0925-4439(92)90023-G

A new, simple assay for long-chain acyl-CoA dehydrogenase in cultured skin fibroblasts using stable isotopes and GC-MS

K. E. Niezen-Koning, R. J. Wanders, G. T. Nagel, L. IJlst, H. S. Heymans

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Abstract

In this paper, we present a new method for measurement of long-chain acyl-CoA dehydrogenase (LCAD) activities in cultured skin fibroblasts. The method is based upon gas chromatographic/mass spectrometric determination of 3-OH-hexadecanoic acid formed during incubation of fibroblasts in a medium containing palmitoyl-CoA and crotonase, to convert the enoyl-CoA ester produced into the 3-hydroxyacyl-CoA ester. The validity of the method is demonstrated by the finding of a full deficiency of LCAD in fibroblasts from three patients with an established deficiency of LCAD

Original language	English
Pages (from-to)	28-32
Journal	Biochimica et Biophysica Acta-Molecular Basis of Disease
Volume	1180
Issue number	1
DOIs	https://doi.org/10.1016/0925-4439(92)90023-G
Publication status	Published - 1992

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https://doi.org/10.1016/0925-4439(92)90023-G

Cite this

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title = "A new, simple assay for long-chain acyl-CoA dehydrogenase in cultured skin fibroblasts using stable isotopes and GC-MS",

abstract = "In this paper, we present a new method for measurement of long-chain acyl-CoA dehydrogenase (LCAD) activities in cultured skin fibroblasts. The method is based upon gas chromatographic/mass spectrometric determination of 3-OH-hexadecanoic acid formed during incubation of fibroblasts in a medium containing palmitoyl-CoA and crotonase, to convert the enoyl-CoA ester produced into the 3-hydroxyacyl-CoA ester. The validity of the method is demonstrated by the finding of a full deficiency of LCAD in fibroblasts from three patients with an established deficiency of LCAD",

author = "Niezen-Koning, {K. E.} and Wanders, {R. J.} and Nagel, {G. T.} and L. IJlst and Heymans, {H. S.}",

year = "1992",

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T1 - A new, simple assay for long-chain acyl-CoA dehydrogenase in cultured skin fibroblasts using stable isotopes and GC-MS

AU - Niezen-Koning, K. E.

AU - Wanders, R. J.

AU - Nagel, G. T.

AU - IJlst, L.

AU - Heymans, H. S.

PY - 1992

Y1 - 1992

N2 - In this paper, we present a new method for measurement of long-chain acyl-CoA dehydrogenase (LCAD) activities in cultured skin fibroblasts. The method is based upon gas chromatographic/mass spectrometric determination of 3-OH-hexadecanoic acid formed during incubation of fibroblasts in a medium containing palmitoyl-CoA and crotonase, to convert the enoyl-CoA ester produced into the 3-hydroxyacyl-CoA ester. The validity of the method is demonstrated by the finding of a full deficiency of LCAD in fibroblasts from three patients with an established deficiency of LCAD

AB - In this paper, we present a new method for measurement of long-chain acyl-CoA dehydrogenase (LCAD) activities in cultured skin fibroblasts. The method is based upon gas chromatographic/mass spectrometric determination of 3-OH-hexadecanoic acid formed during incubation of fibroblasts in a medium containing palmitoyl-CoA and crotonase, to convert the enoyl-CoA ester produced into the 3-hydroxyacyl-CoA ester. The validity of the method is demonstrated by the finding of a full deficiency of LCAD in fibroblasts from three patients with an established deficiency of LCAD

U2 - https://doi.org/10.1016/0925-4439(92)90023-G

DO - https://doi.org/10.1016/0925-4439(92)90023-G

M3 - Article

C2 - 1390941

SN - 0925-4439

VL - 1180

SP - 28

EP - 32

JO - Biochimica et Biophysica Acta-Molecular Basis of Disease

JF - Biochimica et Biophysica Acta-Molecular Basis of Disease

IS - 1

ER -