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Carnitine supplementation induces long-chain acylcarnitine production—Studies in the VLCAD-deficient mouse

  • SSIEM SYMPOSIUM 2005
  • Published:
Journal of Inherited Metabolic Disease

Summary

Carnitine supplementation does not affect carnitine concentrations in tissues of wild-type and very long-chain acyl-CoA dehydrogenase-deficient mice, but results in an increase in long-chain acylcarnitine production.

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References

  • Andresen BS, Olpin S, Poorthuis BJHM, et al (1999) Clear correlation of genotype with disease phenotype in very long-chain acyl-CoA dehydrogenase deficiency. Am J Hum Genet 64: 479–494.

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  • Spiekerkoetter U, Tokunaga C, Wendel U, et al (2005) Tissue carnitine homeostasis in very-long-chain acyl-CoA dehydrogenase-deficient mice. Pediatr Res 57: 760–764.

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Author information

Authors and Affiliations

  1. Department of General Pediatrics, University Children's Hospital, Duesseldorf, Germany

    M. Liebig, M. Gyenes, G. Brauers, U. Wendel, E. Mayatepek & U. Spiekerkoetter

  2. Laboratory of Genetic Metabolic Diseases, Academic Medical Center, Amsterdam, The Netherlands

    J. P. N. Ruiter & R. J. A. Wanders

  3. Vanderbilt University, Nashville, Tennessee, USA

    A. W. Strauss

Authors
  1. M. Liebig
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  2. M. Gyenes
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  3. G. Brauers
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  4. J. P. N. Ruiter
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  5. U. Wendel
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  6. E. Mayatepek
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  7. A. W. Strauss
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  8. R. J. A. Wanders
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  9. U. Spiekerkoetter
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Additional information

Communicating editor: Jean-Marie Saudubray

Competing interests: None declared

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Liebig, M., Gyenes, M., Brauers, G. et al. Carnitine supplementation induces long-chain acylcarnitine production—Studies in the VLCAD-deficient mouse. J Inherit Metab Dis 29, 343–344 (2006). https://doi.org/10.1007/s10545-006-0249-4

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  • DOI: https://doi.org/10.1007/s10545-006-0249-4

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