Effect of heat stress and bezafibrate on mitochondrial beta-oxidation: comparison between cultured cells from normal and mitochondrial fatty acid oxidation disorder children using in vitro probe acylcarnitine profiling assay.
Identifieur interne : 000331 ( Ncbi/Curation ); précédent : 000330; suivant : 000332Effect of heat stress and bezafibrate on mitochondrial beta-oxidation: comparison between cultured cells from normal and mitochondrial fatty acid oxidation disorder children using in vitro probe acylcarnitine profiling assay.
Auteurs : Hong Li [Japon] ; Seiji Fukuda ; Yuki Hasegawa ; Hironori Kobayashi ; Jamiyan Purevsuren ; Yuichi Mushimoto ; Seiji YamaguchiSource :
- Brain & development [ 1872-7131 ] ; 2010.
Descripteurs français
- KwdFr :
- Acides gras (), Acides gras (métabolisme), Acyl-CoA dehydrogenase (déficit), Bézafibrate (pharmacologie), Carnitine (), Carnitine (analogues et dérivés), Carnitine (métabolisme), Cellules cultivées, Enfant, Erreurs innées du métabolisme lipidique (génétique), Erreurs innées du métabolisme lipidique (métabolisme), Fibroblastes (), Fibroblastes (cytologie), Fibroblastes (métabolisme), Humains, Hypolipémiants (pharmacologie), Long-chain-acyl-CoA dehydrogenase (déficit), Maladies mitochondriales (génétique), Maladies mitochondriales (métabolisme), Mitochondries (), Mitochondries (métabolisme), Oxydoréduction (), Stress physiologique, Température élevée.
- MESH :
- analogues et dérivés : Carnitine.
- cytologie : Fibroblastes.
- déficit : Acyl-CoA dehydrogenase, Long-chain-acyl-CoA dehydrogenase.
- génétique : Erreurs innées du métabolisme lipidique, Maladies mitochondriales.
- métabolisme : Acides gras, Carnitine, Erreurs innées du métabolisme lipidique, Fibroblastes, Maladies mitochondriales, Mitochondries.
- pharmacologie : Bézafibrate, Hypolipémiants.
- Acides gras, Carnitine, Cellules cultivées, Enfant, Fibroblastes, Humains, Mitochondries, Oxydoréduction, Stress physiologique, Température élevée.
English descriptors
- KwdEn :
- Acyl-CoA Dehydrogenase (deficiency), Acyl-CoA Dehydrogenase, Long-Chain (deficiency), Bezafibrate (pharmacology), Carnitine (analogs & derivatives), Carnitine (chemistry), Carnitine (metabolism), Cells, Cultured, Child, Fatty Acids (chemistry), Fatty Acids (metabolism), Fibroblasts (cytology), Fibroblasts (drug effects), Fibroblasts (metabolism), Hot Temperature, Humans, Hypolipidemic Agents (pharmacology), Lipid Metabolism, Inborn Errors (genetics), Lipid Metabolism, Inborn Errors (metabolism), Mitochondria (drug effects), Mitochondria (metabolism), Mitochondrial Diseases (genetics), Mitochondrial Diseases (metabolism), Oxidation-Reduction (drug effects), Stress, Physiological.
- MESH :
- chemical , analogs & derivatives : Carnitine.
- chemical , chemistry : Carnitine, Fatty Acids.
- chemical , deficiency : Acyl-CoA Dehydrogenase, Acyl-CoA Dehydrogenase, Long-Chain.
- chemical , metabolism : Carnitine, Fatty Acids.
- chemical , pharmacology : Bezafibrate, Hypolipidemic Agents.
- cytology : Fibroblasts.
- drug effects : Fibroblasts, Mitochondria, Oxidation-Reduction.
- genetics : Lipid Metabolism, Inborn Errors, Mitochondrial Diseases.
- metabolism : Fibroblasts, Lipid Metabolism, Inborn Errors, Mitochondria, Mitochondrial Diseases.
- Cells, Cultured, Child, Hot Temperature, Humans, Stress, Physiological.
Abstract
Hyperpyrexia occasionally triggers acute life-threatening encephalopathy-like illnesses, including influenza-associated encephalopathy (IAE) in childhood, and can be responsible for impaired fatty acid beta-oxidation (FAO). In this regard, patients with impaired FAO may be more susceptible to febrile episodes. The effects of heat stress and a hypolipidemic drug, bezafibrate, on mitochondrial FAO were investigated using cultured cells from children with FAO disorders and from normal controls, using an in vitro probe acylcarnitine (AC) profiling assay. Fibroblasts were incubated in medium loaded with unlabelled palmitic acid for 96 h at 37 and 41 degrees C, with or without bezafibrate. AC profiles in culture medium were analyzed by electrospray ionization tandem mass spectrometry. Heat stress, introduced by 41 degrees C, significantly increased acetylcarnitine (C2) but slightly decreased the other acylcarnitines (ACs) in controls and medium-chain acyl-CoA dehydrogenase (MCAD)-deficient cells. On the other hand, in very long-chain acyl-CoA dehydrogenase (VLCAD)-deficient cells, accumulation of long-chain ACs were enhanced at 41 degrees C, compared with that at 37 degrees C. In contrast, bezafibrate decreased long-chain ACs with significant increase of C2 in both control and VLCAD-deficient cells at 37 degrees C. These data suggest that heat stress specifically inhibits long-chain FAO, whereas bezafibrate recovers the impaired FAO. Our approach is a simple and promising strategy to evaluate the effects of heat stress or therapeutic drugs on mitochondrial FAO.
DOI: 10.1016/j.braindev.2009.06.001
PubMed: 19589653
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pubmed:19589653Le document en format XML
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<term>Bezafibrate (pharmacology)</term>
<term>Carnitine (analogs & derivatives)</term>
<term>Carnitine (chemistry)</term>
<term>Carnitine (metabolism)</term>
<term>Cells, Cultured</term>
<term>Child</term>
<term>Fatty Acids (chemistry)</term>
<term>Fatty Acids (metabolism)</term>
<term>Fibroblasts (cytology)</term>
<term>Fibroblasts (drug effects)</term>
<term>Fibroblasts (metabolism)</term>
<term>Hot Temperature</term>
<term>Humans</term>
<term>Hypolipidemic Agents (pharmacology)</term>
<term>Lipid Metabolism, Inborn Errors (genetics)</term>
<term>Lipid Metabolism, Inborn Errors (metabolism)</term>
<term>Mitochondria (drug effects)</term>
<term>Mitochondria (metabolism)</term>
<term>Mitochondrial Diseases (genetics)</term>
<term>Mitochondrial Diseases (metabolism)</term>
<term>Oxidation-Reduction (drug effects)</term>
<term>Stress, Physiological</term>
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<term>Acides gras (métabolisme)</term>
<term>Acyl-CoA dehydrogenase (déficit)</term>
<term>Bézafibrate (pharmacologie)</term>
<term>Carnitine ()</term>
<term>Carnitine (analogues et dérivés)</term>
<term>Carnitine (métabolisme)</term>
<term>Cellules cultivées</term>
<term>Enfant</term>
<term>Erreurs innées du métabolisme lipidique (génétique)</term>
<term>Erreurs innées du métabolisme lipidique (métabolisme)</term>
<term>Fibroblastes ()</term>
<term>Fibroblastes (cytologie)</term>
<term>Fibroblastes (métabolisme)</term>
<term>Humains</term>
<term>Hypolipémiants (pharmacologie)</term>
<term>Long-chain-acyl-CoA dehydrogenase (déficit)</term>
<term>Maladies mitochondriales (génétique)</term>
<term>Maladies mitochondriales (métabolisme)</term>
<term>Mitochondries ()</term>
<term>Mitochondries (métabolisme)</term>
<term>Oxydoréduction ()</term>
<term>Stress physiologique</term>
<term>Température élevée</term>
</keywords>
<keywords scheme="MESH" type="chemical" qualifier="analogs & derivatives" xml:lang="en"><term>Carnitine</term>
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<term>Fatty Acids</term>
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<term>Acyl-CoA Dehydrogenase, Long-Chain</term>
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<term>Fatty Acids</term>
</keywords>
<keywords scheme="MESH" type="chemical" qualifier="pharmacology" xml:lang="en"><term>Bezafibrate</term>
<term>Hypolipidemic Agents</term>
</keywords>
<keywords scheme="MESH" qualifier="analogues et dérivés" xml:lang="fr"><term>Carnitine</term>
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<keywords scheme="MESH" qualifier="cytologie" xml:lang="fr"><term>Fibroblastes</term>
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<keywords scheme="MESH" qualifier="cytology" xml:lang="en"><term>Fibroblasts</term>
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<keywords scheme="MESH" qualifier="drug effects" xml:lang="en"><term>Fibroblasts</term>
<term>Mitochondria</term>
<term>Oxidation-Reduction</term>
</keywords>
<keywords scheme="MESH" qualifier="déficit" xml:lang="fr"><term>Acyl-CoA dehydrogenase</term>
<term>Long-chain-acyl-CoA dehydrogenase</term>
</keywords>
<keywords scheme="MESH" qualifier="genetics" xml:lang="en"><term>Lipid Metabolism, Inborn Errors</term>
<term>Mitochondrial Diseases</term>
</keywords>
<keywords scheme="MESH" qualifier="génétique" xml:lang="fr"><term>Erreurs innées du métabolisme lipidique</term>
<term>Maladies mitochondriales</term>
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<keywords scheme="MESH" qualifier="metabolism" xml:lang="en"><term>Fibroblasts</term>
<term>Lipid Metabolism, Inborn Errors</term>
<term>Mitochondria</term>
<term>Mitochondrial Diseases</term>
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<term>Carnitine</term>
<term>Erreurs innées du métabolisme lipidique</term>
<term>Fibroblastes</term>
<term>Maladies mitochondriales</term>
<term>Mitochondries</term>
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<term>Hypolipémiants</term>
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<term>Child</term>
<term>Hot Temperature</term>
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<term>Carnitine</term>
<term>Cellules cultivées</term>
<term>Enfant</term>
<term>Fibroblastes</term>
<term>Humains</term>
<term>Mitochondries</term>
<term>Oxydoréduction</term>
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<front><div type="abstract" xml:lang="en">Hyperpyrexia occasionally triggers acute life-threatening encephalopathy-like illnesses, including influenza-associated encephalopathy (IAE) in childhood, and can be responsible for impaired fatty acid beta-oxidation (FAO). In this regard, patients with impaired FAO may be more susceptible to febrile episodes. The effects of heat stress and a hypolipidemic drug, bezafibrate, on mitochondrial FAO were investigated using cultured cells from children with FAO disorders and from normal controls, using an in vitro probe acylcarnitine (AC) profiling assay. Fibroblasts were incubated in medium loaded with unlabelled palmitic acid for 96 h at 37 and 41 degrees C, with or without bezafibrate. AC profiles in culture medium were analyzed by electrospray ionization tandem mass spectrometry. Heat stress, introduced by 41 degrees C, significantly increased acetylcarnitine (C2) but slightly decreased the other acylcarnitines (ACs) in controls and medium-chain acyl-CoA dehydrogenase (MCAD)-deficient cells. On the other hand, in very long-chain acyl-CoA dehydrogenase (VLCAD)-deficient cells, accumulation of long-chain ACs were enhanced at 41 degrees C, compared with that at 37 degrees C. In contrast, bezafibrate decreased long-chain ACs with significant increase of C2 in both control and VLCAD-deficient cells at 37 degrees C. These data suggest that heat stress specifically inhibits long-chain FAO, whereas bezafibrate recovers the impaired FAO. Our approach is a simple and promising strategy to evaluate the effects of heat stress or therapeutic drugs on mitochondrial FAO.</div>
</front>
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