Inhibition of oxidative phosphorylation by palmitoyl-CoA in digitonin permeabilized fibroblasts: implications for long-chain fatty acid beta-oxidation disorders

F. V. Ventura; J. P. Ruiter; L. IJlst; I. T. Almeida; R. J. Wanders

doi:https://doi.org/10.1016/0925-4439(95)00064-B

Inhibition of oxidative phosphorylation by palmitoyl-CoA in digitonin permeabilized fibroblasts: implications for long-chain fatty acid beta-oxidation disorders

F. V. Ventura, J. P. Ruiter, L. IJlst, I. T. Almeida, R. J. Wanders

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Research output: Contribution to journal › Article › Academic › peer-review

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Abstract

Long-chain fatty acid oxidation deficient patients present early in life with more severe features than patients with a medium-chain fatty acid oxidation deficiency. This may be related to the more toxic effect of long-chain fatty acid derivatives. In this paper we have studied the effect of different acyl-CoA esters, and palmitoyl-CoA in particular, on succinate-driven oxidative phosphorylation, using digitonin permeabilized human fibroblasts. Palmitoyl-CoA was found to inhibit the succinate-driven oxidative phosphorylation in a concentration dependent manner. If the inhibition of the oxidative phosphorylation system is also expressed under in vivo conditions this might explain some of the abnormalities found in patients with defects in long-chain fatty acid beta-oxidation

Original language	English
Pages (from-to)	14-20
Journal	Biochimica et Biophysica Acta-Molecular Basis of Disease
Volume	1272
Issue number	1
DOIs	https://doi.org/10.1016/0925-4439(95)00064-B
Publication status	Published - 1995

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https://doi.org/10.1016/0925-4439(95)00064-B

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@article{b1ee95fb75734bda98f8caf2a3d2c8ea,

title = "Inhibition of oxidative phosphorylation by palmitoyl-CoA in digitonin permeabilized fibroblasts: implications for long-chain fatty acid beta-oxidation disorders",

abstract = "Long-chain fatty acid oxidation deficient patients present early in life with more severe features than patients with a medium-chain fatty acid oxidation deficiency. This may be related to the more toxic effect of long-chain fatty acid derivatives. In this paper we have studied the effect of different acyl-CoA esters, and palmitoyl-CoA in particular, on succinate-driven oxidative phosphorylation, using digitonin permeabilized human fibroblasts. Palmitoyl-CoA was found to inhibit the succinate-driven oxidative phosphorylation in a concentration dependent manner. If the inhibition of the oxidative phosphorylation system is also expressed under in vivo conditions this might explain some of the abnormalities found in patients with defects in long-chain fatty acid beta-oxidation",

author = "Ventura, {F. V.} and Ruiter, {J. P.} and L. IJlst and Almeida, {I. T.} and Wanders, {R. J.}",

year = "1995",

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volume = "1272",

pages = "14--20",

journal = "Biochimica et Biophysica Acta-Molecular Basis of Disease",

issn = "0925-4439",

publisher = "Elsevier",

number = "1",

}

Inhibition of oxidative phosphorylation by palmitoyl-CoA in digitonin permeabilized fibroblasts: implications for long-chain fatty acid beta-oxidation disorders. / Ventura, F. V.; Ruiter, J. P.; IJlst, L. et al.
In: Biochimica et Biophysica Acta-Molecular Basis of Disease, Vol. 1272, No. 1, 1995, p. 14-20.

Research output: Contribution to journal › Article › Academic › peer-review

TY - JOUR

T1 - Inhibition of oxidative phosphorylation by palmitoyl-CoA in digitonin permeabilized fibroblasts: implications for long-chain fatty acid beta-oxidation disorders

AU - Ventura, F. V.

AU - Ruiter, J. P.

AU - IJlst, L.

AU - Almeida, I. T.

AU - Wanders, R. J.

PY - 1995

Y1 - 1995

N2 - Long-chain fatty acid oxidation deficient patients present early in life with more severe features than patients with a medium-chain fatty acid oxidation deficiency. This may be related to the more toxic effect of long-chain fatty acid derivatives. In this paper we have studied the effect of different acyl-CoA esters, and palmitoyl-CoA in particular, on succinate-driven oxidative phosphorylation, using digitonin permeabilized human fibroblasts. Palmitoyl-CoA was found to inhibit the succinate-driven oxidative phosphorylation in a concentration dependent manner. If the inhibition of the oxidative phosphorylation system is also expressed under in vivo conditions this might explain some of the abnormalities found in patients with defects in long-chain fatty acid beta-oxidation

AB - Long-chain fatty acid oxidation deficient patients present early in life with more severe features than patients with a medium-chain fatty acid oxidation deficiency. This may be related to the more toxic effect of long-chain fatty acid derivatives. In this paper we have studied the effect of different acyl-CoA esters, and palmitoyl-CoA in particular, on succinate-driven oxidative phosphorylation, using digitonin permeabilized human fibroblasts. Palmitoyl-CoA was found to inhibit the succinate-driven oxidative phosphorylation in a concentration dependent manner. If the inhibition of the oxidative phosphorylation system is also expressed under in vivo conditions this might explain some of the abnormalities found in patients with defects in long-chain fatty acid beta-oxidation

U2 - https://doi.org/10.1016/0925-4439(95)00064-B

DO - https://doi.org/10.1016/0925-4439(95)00064-B

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C2 - 7662716

SN - 0925-4439

VL - 1272

SP - 14

EP - 20

JO - Biochimica et Biophysica Acta-Molecular Basis of Disease

JF - Biochimica et Biophysica Acta-Molecular Basis of Disease

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