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Literature DB >> 16763898

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

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

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.

Entities: Chemical Gene Species

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Year: 2006 PMID： 16763898 DOI： 10.1007/s10545-006-0249-4

Source DB: PubMed Journal: J Inherit Metab Dis ISSN： 0141-8955 Impact factor: 4.982

2 in total

1. Tissue carnitine homeostasis in very-long-chain acyl-CoA dehydrogenase-deficient mice.

Authors: Ute Spiekerkoetter; Chonan Tokunaga; Udo Wendel; Ertan Mayatepek; Lodewijk Ijlst; Frederic M Vaz; Naomi van Vlies; Henk Overmars; Marinus Duran; Frits A Wijburg; Ronald J Wanders; Arnold W Strauss
Journal: Pediatr Res Date: 2005-03-17 Impact factor: 3.756

2. Clear correlation of genotype with disease phenotype in very-long-chain acyl-CoA dehydrogenase deficiency.

Authors: B S Andresen; S Olpin; B J Poorthuis; H R Scholte; C Vianey-Saban; R Wanders; L Ijlst; A Morris; M Pourfarzam; K Bartlett; E R Baumgartner; J B deKlerk; L D Schroeder; T J Corydon; H Lund; V Winter; P Bross; L Bolund; N Gregersen
Journal: Am J Hum Genet Date: 1999-02 Impact factor: 11.025

2 in total

7 in total

1. Treatment recommendations in long-chain fatty acid oxidation defects: consensus from a workshop.

Authors: U Spiekerkoetter; M Lindner; R Santer; M Grotzke; M R Baumgartner; H Boehles; A Das; C Haase; J B Hennermann; D Karall; H de Klerk; I Knerr; H G Koch; B Plecko; W Röschinger; K O Schwab; D Scheible; F A Wijburg; J Zschocke; E Mayatepek; U Wendel
Journal: J Inherit Metab Dis Date: 2009-04-28 Impact factor: 4.982

2. Corresponding increase in long-chain acyl-CoA and acylcarnitine after exercise in muscle from VLCAD mice.

Authors: Frank ter Veld; Sonja Primassin; Lars Hoffmann; Ertan Mayatepek; Ute Spiekerkoetter
Journal: J Lipid Res Date: 2008-10-31 Impact factor: 5.922

3. Management and outcome in 75 individuals with long-chain fatty acid oxidation defects: results from a workshop.

4. Carnitine supplementation attenuates myocardial lipid accumulation in long-chain acyl-CoA dehydrogenase knockout mice.

Authors: Adrianus J Bakermans; Michel van Weeghel; Simone Denis; Klaas Nicolay; Jeanine J Prompers; Sander M Houten
Journal: J Inherit Metab Dis Date: 2013-04-06 Impact factor: 4.982

5. A Delphi clinical practice protocol for the management of very long chain acyl-CoA dehydrogenase deficiency.

Authors: Georgianne L Arnold; Johan Van Hove; Debra Freedenberg; Arnold Strauss; Nicola Longo; Barbara Burton; Cheryl Garganta; Can Ficicioglu; Stephen Cederbaum; Cary Harding; Richard G Boles; Dietrich Matern; Pranesh Chakraborty; Annette Feigenbaum
Journal: Mol Genet Metab Date: 2009-01-20 Impact factor: 4.797

6. Functional effects of different medium-chain acyl-CoA dehydrogenase genotypes and identification of asymptomatic variants.

Authors: Marga Sturm; Diran Herebian; Martina Mueller; Maurice D Laryea; Ute Spiekerkoetter
Journal: PLoS One Date: 2012-09-17 Impact factor: 3.240

7. Two siblings with very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency suffered from rhabdomyolysis after l-carnitine supplementation.

Authors: Kenji Watanabe; Kenji Yamada; Koji Sameshima; Seiji Yamaguchi
Journal: Mol Genet Metab Rep Date: 2018-04-13

7 in total