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

doi:10.1007/s10545-006-0249-4

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

J Inherit Metab Dis. 2006 Apr-Jun;29(2-3):343-4. doi: 10.1007/s10545-006-0249-4.

Authors

M Liebig¹, M Gyenes, G Brauers, J P N Ruiter, U Wendel, E Mayatepek, A W Strauss, R J A Wanders, U Spiekerkoetter

Affiliation

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

PMID: 16763898
DOI: 10.1007/s10545-006-0249-4

Abstract

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.

MeSH terms

Acyl-CoA Dehydrogenase, Long-Chain / deficiency*
Acyl-CoA Dehydrogenase, Long-Chain / genetics
Acyl-CoA Dehydrogenase, Long-Chain / metabolism
Animals
Carnitine / analogs & derivatives*
Carnitine / blood
Carnitine / metabolism
Carnitine / pharmacology*
Carnitine / therapeutic use
Dietary Supplements*
Disease Models, Animal
Homeostasis
Lipid Metabolism, Inborn Errors / drug therapy*
Lipid Metabolism, Inborn Errors / metabolism
Liver / metabolism
Mice
Mice, Knockout
Muscle, Skeletal / metabolism
Myocardium / metabolism
Physical Conditioning, Animal

Substances

acylcarnitine
Acyl-CoA Dehydrogenase, Long-Chain
Carnitine