Tissue acylcarnitine status in a mouse model of mitochondrial β-oxidation deficiency during metabolic decompensation due to influenza virus infection.
Identifieur interne : 000949 ( Main/Exploration ); précédent : 000948; suivant : 000950Tissue acylcarnitine status in a mouse model of mitochondrial β-oxidation deficiency during metabolic decompensation due to influenza virus infection.
Auteurs : Tatiana N. Tarasenko [États-Unis] ; Kristina Cusmano-Ozog [États-Unis] ; Peter J. Mcguire [États-Unis]Source :
- Molecular genetics and metabolism [ 1096-7206 ] ; 2018.
Descripteurs français
- KwdFr :
- Acides gras (métabolisme), Animaux, Cardiomyopathies (anatomopathologie), Cardiomyopathies (génétique), Cardiomyopathies (métabolisme), Carnitine (analogues et dérivés), Carnitine (métabolisme), Défaillance hépatique (anatomopathologie), Défaillance hépatique (génétique), Défaillance hépatique (métabolisme), Erreurs innées du métabolisme lipidique (génétique), Femelle, Foie (métabolisme), Foie (physiologie), Humains, Hypoglycémie (anatomopathologie), Hypoglycémie (génétique), Hypoglycémie (métabolisme), Long-chain-acyl-CoA dehydrogenase (déficit), Long-chain-acyl-CoA dehydrogenase (génétique), Maladies mitochondriales (génétique), Maladies musculaires (génétique), Maladies métaboliques (anatomopathologie), Maladies métaboliques (génétique), Maladies métaboliques (métabolisme), Modèles animaux de maladie humaine, Muscles squelettiques (anatomopathologie), Muscles squelettiques (métabolisme), Myocarde (anatomopathologie), Oxydoréduction, Peroxydation lipidique (génétique), Souris.
- MESH :
- analogues et dérivés : Carnitine.
- anatomopathologie : Cardiomyopathies, Défaillance hépatique, Hypoglycémie, Maladies métaboliques, Muscles squelettiques, Myocarde.
- déficit : Long-chain-acyl-CoA dehydrogenase.
- génétique : Cardiomyopathies, Défaillance hépatique, Erreurs innées du métabolisme lipidique, Hypoglycémie, Long-chain-acyl-CoA dehydrogenase, Maladies mitochondriales, Maladies musculaires, Maladies métaboliques, Peroxydation lipidique.
- métabolisme : Acides gras, Cardiomyopathies, Carnitine, Défaillance hépatique, Foie, Hypoglycémie, Maladies métaboliques, Muscles squelettiques.
- physiologie : Foie.
- Animaux, Femelle, Humains, Modèles animaux de maladie humaine, Oxydoréduction, Souris.
English descriptors
- KwdEn :
- Acyl-CoA Dehydrogenase, Long-Chain (deficiency), Acyl-CoA Dehydrogenase, Long-Chain (genetics), Animals, Cardiomyopathies (genetics), Cardiomyopathies (metabolism), Cardiomyopathies (pathology), Carnitine (analogs & derivatives), Carnitine (metabolism), Congenital Bone Marrow Failure Syndromes, Disease Models, Animal, Fatty Acids (metabolism), Female, Humans, Hypoglycemia (genetics), Hypoglycemia (metabolism), Hypoglycemia (pathology), Lipid Metabolism, Inborn Errors (genetics), Lipid Peroxidation (genetics), Liver (metabolism), Liver (physiology), Liver Failure (genetics), Liver Failure (metabolism), Liver Failure (pathology), Metabolic Diseases (genetics), Metabolic Diseases (metabolism), Metabolic Diseases (pathology), Mice, Mitochondrial Diseases (genetics), Muscle, Skeletal (metabolism), Muscle, Skeletal (pathology), Muscular Diseases (genetics), Myocardium (pathology), Oxidation-Reduction.
- MESH :
- chemical , analogs & derivatives : Carnitine.
- chemical , deficiency : Acyl-CoA Dehydrogenase, Long-Chain.
- chemical , genetics : Acyl-CoA Dehydrogenase, Long-Chain.
- genetics : Cardiomyopathies, Hypoglycemia, Lipid Metabolism, Inborn Errors, Lipid Peroxidation, Liver Failure, Metabolic Diseases, Mitochondrial Diseases, Muscular Diseases.
- metabolism : Cardiomyopathies, Carnitine, Fatty Acids, Hypoglycemia, Liver, Liver Failure, Metabolic Diseases, Muscle, Skeletal.
- pathology : Cardiomyopathies, Hypoglycemia, Liver Failure, Metabolic Diseases, Muscle, Skeletal, Myocardium.
- physiology : Liver.
- Animals, Congenital Bone Marrow Failure Syndromes, Disease Models, Animal, Female, Humans, Mice, Oxidation-Reduction.
Abstract
Despite judicious monitoring and care, patients with fatty acid oxidation disorders may experience metabolic decompensation due to infection which may result in rhabdomyolysis, cardiomyopathy, hypoglycemia and liver dysfunction and failure. Since clinical studies on metabolic decompensation are dangerous, we employed a preclinical model of metabolic decompensation due to infection. By infecting mice with mouse adapted influenza and using a pair-feeding strategy in a mouse model of long-chain fatty acid oxidation (Acadvl-/-), our goals were to isolate the effects of infection on tissue acylcarnitines and determine how they relate to their plasma counterparts. Applying statistical data reduction techniques (Partial Least Squares-Discriminant Analysis), we were able to identify critical acylcarnitines that were driving differentiation of our experimental groups for all the tissues studied. While plasma displayed increases in metabolites directly related to mouse VLCAD deficiency (e.g. C16 and C18), organs like the heart, muscle and liver also showed involvement of alternative pathways (e.g. medium-chain FAO and ketogenesis), suggesting adaptive measures. Matched correlation analyses showed strong correlations (r > 0.7) between plasma and tissue levels for a small number of metabolites. Overall, our results demonstrate that infection as a stress produces perturbations in metabolism in Acadvl-/- that differ greatly from WT infected and Acadvl-/- pair-fed controls. This model system will be useful for studying the effects of infection on tissue metabolism as well as evaluating interventions aimed at modulating the effects of metabolic decompensation.
DOI: 10.1016/j.ymgme.2018.06.012
PubMed: 30031688
Affiliations:
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Failure Syndromes</term><term>Disease Models, Animal</term><term>Fatty Acids (metabolism)</term><term>Female</term><term>Humans</term><term>Hypoglycemia (genetics)</term><term>Hypoglycemia (metabolism)</term><term>Hypoglycemia (pathology)</term><term>Lipid Metabolism, Inborn Errors (genetics)</term><term>Lipid Peroxidation (genetics)</term><term>Liver (metabolism)</term><term>Liver (physiology)</term><term>Liver Failure (genetics)</term><term>Liver Failure (metabolism)</term><term>Liver Failure (pathology)</term><term>Metabolic Diseases (genetics)</term><term>Metabolic Diseases (metabolism)</term><term>Metabolic Diseases (pathology)</term><term>Mice</term><term>Mitochondrial Diseases (genetics)</term><term>Muscle, Skeletal (metabolism)</term><term>Muscle, Skeletal (pathology)</term><term>Muscular Diseases (genetics)</term><term>Myocardium (pathology)</term><term>Oxidation-Reduction</term></keywords><keywords scheme="KwdFr" xml:lang="fr"><term>Acides gras 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(génétique)</term><term>Maladies métaboliques (métabolisme)</term><term>Modèles animaux de maladie humaine</term><term>Muscles squelettiques (anatomopathologie)</term><term>Muscles squelettiques (métabolisme)</term><term>Myocarde (anatomopathologie)</term><term>Oxydoréduction</term><term>Peroxydation lipidique (génétique)</term><term>Souris</term></keywords><keywords scheme="MESH" type="chemical" qualifier="analogs & derivatives" xml:lang="en"><term>Carnitine</term></keywords><keywords scheme="MESH" type="chemical" qualifier="deficiency" xml:lang="en"><term>Acyl-CoA Dehydrogenase, Long-Chain</term></keywords><keywords scheme="MESH" type="chemical" qualifier="genetics" xml:lang="en"><term>Acyl-CoA Dehydrogenase, Long-Chain</term></keywords><keywords scheme="MESH" qualifier="analogues et dérivés" xml:lang="fr"><term>Carnitine</term></keywords><keywords scheme="MESH" qualifier="anatomopathologie" xml:lang="fr"><term>Cardiomyopathies</term><term>Défaillance 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qualifier="metabolism" xml:lang="en"><term>Cardiomyopathies</term><term>Carnitine</term><term>Fatty Acids</term><term>Hypoglycemia</term><term>Liver</term><term>Liver Failure</term><term>Metabolic Diseases</term><term>Muscle, Skeletal</term></keywords><keywords scheme="MESH" qualifier="métabolisme" xml:lang="fr"><term>Acides gras</term><term>Cardiomyopathies</term><term>Carnitine</term><term>Défaillance hépatique</term><term>Foie</term><term>Hypoglycémie</term><term>Maladies métaboliques</term><term>Muscles squelettiques</term></keywords><keywords scheme="MESH" qualifier="pathology" xml:lang="en"><term>Cardiomyopathies</term><term>Hypoglycemia</term><term>Liver Failure</term><term>Metabolic Diseases</term><term>Muscle, Skeletal</term><term>Myocardium</term></keywords><keywords scheme="MESH" qualifier="physiologie" xml:lang="fr"><term>Foie</term></keywords><keywords scheme="MESH" qualifier="physiology" xml:lang="en"><term>Liver</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Animals</term><term>Congenital Bone Marrow Failure Syndromes</term><term>Disease Models, Animal</term><term>Female</term><term>Humans</term><term>Mice</term><term>Oxidation-Reduction</term></keywords><keywords scheme="MESH" xml:lang="fr"><term>Animaux</term><term>Femelle</term><term>Humains</term><term>Modèles animaux de maladie humaine</term><term>Oxydoréduction</term><term>Souris</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Despite judicious monitoring and care, patients with fatty acid oxidation disorders may experience metabolic decompensation due to infection which may result in rhabdomyolysis, cardiomyopathy, hypoglycemia and liver dysfunction and failure. Since clinical studies on metabolic decompensation are dangerous, we employed a preclinical model of metabolic decompensation due to infection. By infecting mice with mouse adapted influenza and using a pair-feeding strategy in a mouse model of long-chain fatty acid oxidation (Acadvl<sup>-/-</sup>), our goals were to isolate the effects of infection on tissue acylcarnitines and determine how they relate to their plasma counterparts. Applying statistical data reduction techniques (Partial Least Squares-Discriminant Analysis), we were able to identify critical acylcarnitines that were driving differentiation of our experimental groups for all the tissues studied. While plasma displayed increases in metabolites directly related to mouse VLCAD deficiency (e.g. C16 and C18), organs like the heart, muscle and liver also showed involvement of alternative pathways (e.g. medium-chain FAO and ketogenesis), suggesting adaptive measures. Matched correlation analyses showed strong correlations (r > 0.7) between plasma and tissue levels for a small number of metabolites. Overall, our results demonstrate that infection as a stress produces perturbations in metabolism in Acadvl<sup>-/-</sup> that differ greatly from WT infected and Acadvl<sup>-/-</sup> pair-fed controls. This model system will be useful for studying the effects of infection on tissue metabolism as well as evaluating interventions aimed at modulating the effects of metabolic decompensation.</div></front></TEI><affiliations><list><country><li>États-Unis</li></country><region><li>District de Columbia</li></region></list><tree><country name="États-Unis"><noRegion><name sortKey="Tarasenko, Tatiana N" sort="Tarasenko, Tatiana N" uniqKey="Tarasenko T" first="Tatiana N" last="Tarasenko">Tatiana N. Tarasenko</name></noRegion><name sortKey="Cusmano Ozog, Kristina" sort="Cusmano Ozog, Kristina" uniqKey="Cusmano Ozog K" first="Kristina" last="Cusmano-Ozog">Kristina Cusmano-Ozog</name><name sortKey="Mcguire, Peter J" sort="Mcguire, Peter J" uniqKey="Mcguire P" first="Peter J" last="Mcguire">Peter J. Mcguire</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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