Document Detail

Myocardial function and energy metabolism in carnitine-deficient rats.
MedLine Citation:
PMID:  12785723     Owner:  NLM     Status:  MEDLINE    
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.
C E Zaugg; M Spaniol; P Kaufmann; M Bellahcene; V Barbosa; M Tolnay; P T Buser; S Krähenbühl
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Cellular and molecular life sciences : CMLS     Volume:  60     ISSN:  1420-682X     ISO Abbreviation:  Cell. Mol. Life Sci.     Publication Date:  2003 Apr 
Date Detail:
Created Date:  2003-06-05     Completed Date:  2003-06-25     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  9705402     Medline TA:  Cell Mol Life Sci     Country:  Switzerland    
Other Details:
Languages:  eng     Pagination:  767-75     Citation Subset:  IM    
Department of Research, University Hospital, Hebelstrasse 20, 4031 Basel, Switzerland.
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MeSH Terms
Adenosine Triphosphate / metabolism
Carnitine / blood,  deficiency*,  metabolism
Glycogen / deficiency,  metabolism
Heart / physiology*
Mitochondria / enzymology
Myocardium / cytology
Phosphocreatine / metabolism
Reg. No./Substance:
541-15-1/Carnitine; 56-65-5/Adenosine Triphosphate; 67-07-2/Phosphocreatine; 9005-79-2/Glycogen

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