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Journal of Inherited Metabolic Disease

, Volume 29, Issue 2–3, pp 343–344 | Cite as

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

  • M. Liebig
  • M. Gyenes
  • G. Brauers
  • J. P. N. Ruiter
  • U. Wendel
  • E. Mayatepek
  • A. W. Strauss
  • R. J. A. Wanders
  • U. Spiekerkoetter
SSIEM SYMPOSIUM 2005

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.

Keywords

Carnitine Heart Muscle Free Carnitine Carnitine Supplementation Carnitine Concentration 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

References

  1. 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.PubMedCrossRefGoogle Scholar
  2. 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.PubMedCrossRefGoogle Scholar

Copyright information

© SSIEM and Springer 2006

Authors and Affiliations

  • M. Liebig
    • 1
  • M. Gyenes
    • 1
  • G. Brauers
    • 1
  • J. P. N. Ruiter
    • 2
  • U. Wendel
    • 1
  • E. Mayatepek
    • 1
  • A. W. Strauss
    • 3
  • R. J. A. Wanders
    • 2
  • U. Spiekerkoetter
    • 1
  1. 1.Department of General PediatricsUniversity Children's HospitalDuesseldorfGermany
  2. 2.Laboratory of Genetic Metabolic DiseasesAcademic Medical CenterAmsterdamThe Netherlands
  3. 3.Vanderbilt UniversityNashvilleUSA

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