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Effect of carnitine deprivation on carnitine homeostasis and energy metabolism in mice with systemic carnitine deficiency

Knapp, Andrea Caroline and Todesco, Liliane and Torok, Michael and Beier, Konstantin and Krähenbühl, Stephan. (2008) Effect of carnitine deprivation on carnitine homeostasis and energy metabolism in mice with systemic carnitine deficiency. Annals of nutrition and metabolism, Vol. 52, No. 2. pp. 136-144.

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Official URL: http://edoc.unibas.ch/dok/A5248826

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Abstract

Background/Aims: Juvenile visceral steatosis (jvs-/-) mice lack the activity of the carnitine transporter OCTN2 and are dependent on carnitine substitution. The effects of carnitine deprivation on carnitine homeostasis and energy metabolism are not known in jvs-/- mice. Methods: jvs-/- mice were studied 3, 6 and 10 days after carnitine deprivation, and compared to jvs-/- mice substituted with carnitine, wildtype (jvs+/+) and jvs+/- mice. Carnitine concentrations were assessed radioenzymatically. Results: Compared to wildtype mice, carnitine- treated jvs-/- mice had decreased plasma beta-hydroxybutyrate levels and showed hepatic fat accumulation. The carnitine levels in plasma, liver and skeletal muscle were decreased by 58, 16 and 17 the plasma carnitine concentration had fallen by 87 and the tissue carnitine levels by approximate to 50 carnitine- treated jvs-/- mice. Carnitine deprivation was associated with a further drop in plasma beta-hydroxybutyrate and increased hepatic fat. Skeletal muscle glycogen stores decreased and lactate levels increased with carnitine deprivation, whereas tissue ATP levels were maintained. Conclusions: In jvs-/- mice, tissue carnitine stores are more resistant than carnitine plasma concentrations to carnitine deprivation. Metabolic changes (liver steatosis and loss of muscle glycogen stores) appear also early after carnitine deprivation. Copyright (C) 2008 S. Karger AG, Basel.
Faculties and Departments:03 Faculty of Medicine > Bereich Medizinische Fächer (Klinik) > Klinische Pharmakologie > Klinische Pharmakologie (Krähenbühl)
03 Faculty of Medicine > Departement Klinische Forschung > Bereich Medizinische Fächer (Klinik) > Klinische Pharmakologie > Klinische Pharmakologie (Krähenbühl)
03 Faculty of Medicine > Departement Biomedizin > Division of Anatomy > Histologie (Beier)
UniBasel Contributors:Krähenbühl, Stephan and Beier, Konstantin
Item Type:Article, refereed
Article Subtype:Research Article
Bibsysno:Link to catalogue
ISSN:0250-6807
Note:Publication type according to Uni Basel Research Database: Journal article
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Last Modified:12 Sep 2014 08:02
Deposited On:22 Mar 2012 13:39

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