Myocardial function and energy metabolism in carnitine-deficient rats

Carnitine is essential for mitochondrial metabolism of long-chain fatty acids and thus for myocardial energy production. Accordingly, carnitine deficiency can be associated with cardiomyopathy. To better understand this disease, we determined myocardial function and energy metabolism in a rat model...

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Bibliographic Details
Published in:Cellular and molecular life sciences : CMLS 2003-04, Vol.60 (4), p.767-775
Main Authors: Zaugg, C E, Spaniol, M, Kaufmann, P, Bellahcene, M, Barbosa, V, Tolnay, M, Buser, P T, Krähenbühl, S
Format: Article
Language:English
Subjects:
Adenosine Triphosphate - metabolism
Animals
Cardiovascular disease
Carnitine - blood
Carnitine - deficiency
Carnitine - metabolism
Fatty acids
Glycogen - deficiency
Glycogen - metabolism
Heart - physiology
Histology
Metabolism
Mitochondria
Mitochondria - enzymology
Molecular biology
Myocardium - cytology
Phosphocreatine - metabolism
Rats
Rodents
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Summary:Carnitine is essential for mitochondrial metabolism of long-chain fatty acids and thus for myocardial energy production. Accordingly, carnitine deficiency can be associated with cardiomyopathy. To better understand this disease, we determined myocardial function and energy metabolism in a rat model of carnitine deficiency. Carnitine deficiency was induced by a 3- or 6-week diet containing N-trimethyl-hydrazine-3-propionate, reducing cardiac and plasma carnitine by 70-85%. Myocardial function was investigated in isolated isovolumic heart preparations. Carnitine-deficient hearts showed left ventricular systolic dysfunction, reduced contractile reserve, and a blunted frequency-force relationship independently of the substrate used (glucose or palmitate). After glycogen depletion, palmitate could not sustain myocardial function. Histology and activities of carnitine palmitoyl transferase, citrate synthase, and cytochrome c oxidase were unaltered. Thus, as little as 3-6 weeks of systemic carnitine deficiency can lead to abnormalities in myocardial function. These abnormalities are masked by endogenous glycogen and are not accompanied by structural alterations of the myocardium or by altered activities of important mitochondrial enzymes.
ISSN:1420-682X
1420-9071
DOI:10.1007/s00018-003-3011-1