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The Carnitine System pp 145–166Cite as

Myocardial carnitine deficiency in human cardiomyopathy

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Part of the book series: Developments in Cardiovascular Medicine ((DICM,volume 162))

Abstract

In the literature an important role for carnitine has been discussed in myocardial ischemia and several non-ischemic heart diseases. The presumably broad spectrum of carnitine effects in the heart is based on the central role of this compound in fatty acid oxidation and in the control of intermediary metabolism [1–3]. Long-chain acyl-CoA esters may only penetrate into the mitochon-drial matrix in the form of their carnitine esters, and intracellular concentrations of long-chain acyl-CoA and long-chain acylcarnitine as well as free CoA depend on the availability of free carnitine. Thus, this availability controls basic cellular functions such as energy production and energy transport from the mitochondria into cytoplasm. The interaction between carnitine and potentially toxic products of fatty acid metabolism, as long-chain acyl-CoA esters, may explain the protection of mitochondrial function and the modulation of the adenine nucleotide translocator activity by carnitine as well as its effects on the integrity and fluidity of sarcolemmal membranes.

“In heart failure, the myocardial carnitine level can decrease to concentrations in the range of the KM of carnitine palmityltransferase for free carnitine. Thus, in these cases a reduced availability of free carnitine may limit the transferase reaction and thereby fatty acid oxidation.”

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Author information

Authors and Affiliations

  1. Department of Cardiology, German Heart Institute Berlin, Augustenburger Platz 1, P.O. Box 650505, D-13305, Berlin, Germany

    Dr Vera Regitz-Zagrosek

Authors
  1. Dr Vera Regitz-Zagrosek
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  2. Eckart Fleck
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Editors and Affiliations

  1. Thoraxcenter, Erasmus University, Rotterdam, The Netherlands

    J. W. De Jong

  2. Cattedra di Cardiologia, Università degli Studi di Brescia, Fondazione Clinica del Lavoro di Pavia, Gussago/Brescia, Italy

    R. Ferrari

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© 1995 Springer Science+Business Media Dordrecht

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Regitz-Zagrosek, V., Fleck, E. (1995). Myocardial carnitine deficiency in human cardiomyopathy. In: De Jong, J.W., Ferrari, R. (eds) The Carnitine System. Developments in Cardiovascular Medicine, vol 162. Springer, Dordrecht. https://doi.org/10.1007/978-94-011-0275-9_11

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  • DOI: https://doi.org/10.1007/978-94-011-0275-9_11

  • Publisher Name: Springer, Dordrecht

  • Print ISBN: 978-94-010-4122-5

  • Online ISBN: 978-94-011-0275-9

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