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Journal Abstract Search

547 related items for PubMed ID: 18317232

  • 1. Carnitine supplementation induces acylcarnitine production in tissues of very long-chain acyl-CoA dehydrogenase-deficient mice, without replenishing low free carnitine.
    Primassin S, Ter Veld F, Mayatepek E, Spiekerkoetter U.
    Pediatr Res; 2008 Jun; 63(6):632-7. PubMed ID: 18317232
    [Abstract] [Full Text] [Related]

  • 2. Tissue carnitine homeostasis in very-long-chain acyl-CoA dehydrogenase-deficient mice.
    Spiekerkoetter U, Tokunaga C, Wendel U, Mayatepek E, Ijlst L, Vaz FM, van Vlies N, Overmars H, Duran M, Wijburg FA, Wanders RJ, Strauss AW.
    Pediatr Res; 2005 Jun; 57(6):760-4. PubMed ID: 15774826
    [Abstract] [Full Text] [Related]

  • 3. Changes in blood carnitine and acylcarnitine profiles of very long-chain acyl-CoA dehydrogenase-deficient mice subjected to stress.
    Spiekerkoetter U, Tokunaga C, Wendel U, Mayatepek E, Exil V, Duran M, Wijburg FA, Wanders RJ, Strauss AW.
    Eur J Clin Invest; 2004 Mar; 34(3):191-6. PubMed ID: 15025677
    [Abstract] [Full Text] [Related]

  • 4. Carnitine supplementation induces long-chain acylcarnitine production--studies in the VLCAD-deficient mouse.
    Liebig M, Gyenes M, Brauers G, Ruiter JP, Wendel U, Mayatepek E, Strauss AW, Wanders RJ, Spiekerkoetter U.
    J Inherit Metab Dis; 2006 Mar; 29(2-3):343-4. PubMed ID: 16763898
    [Abstract] [Full Text] [Related]

  • 5. Neonatal screening for very long-chain acyl-coA dehydrogenase deficiency: enzymatic and molecular evaluation of neonates with elevated C14:1-carnitine levels.
    Liebig M, Schymik I, Mueller M, Wendel U, Mayatepek E, Ruiter J, Strauss AW, Wanders RJ, Spiekerkoetter U.
    Pediatrics; 2006 Sep; 118(3):1065-9. PubMed ID: 16950999
    [Abstract] [Full Text] [Related]

  • 6. Bezafibrate increases very-long-chain acyl-CoA dehydrogenase protein and mRNA expression in deficient fibroblasts and is a potential therapy for fatty acid oxidation disorders.
    Djouadi F, Aubey F, Schlemmer D, Ruiter JP, Wanders RJ, Strauss AW, Bastin J.
    Hum Mol Genet; 2005 Sep 15; 14(18):2695-703. PubMed ID: 16115821
    [Abstract] [Full Text] [Related]

  • 7. Hepatic and muscular effects of different dietary fat content in VLCAD deficient mice.
    Primassin S, Tucci S, Spiekerkoetter U.
    Mol Genet Metab; 2011 Dec 15; 104(4):546-51. PubMed ID: 21963783
    [Abstract] [Full Text] [Related]

  • 8. Pre-exercise medium-chain triglyceride application prevents acylcarnitine accumulation in skeletal muscle from very-long-chain acyl-CoA-dehydrogenase-deficient mice.
    Primassin S, Tucci S, Herebian D, Seibt A, Hoffmann L, ter Veld F, Spiekerkoetter U.
    J Inherit Metab Dis; 2010 Jun 15; 33(3):237-46. PubMed ID: 20446112
    [Abstract] [Full Text] [Related]

  • 9. Corresponding increase in long-chain acyl-CoA and acylcarnitine after exercise in muscle from VLCAD mice.
    ter Veld F, Primassin S, Hoffmann L, Mayatepek E, Spiekerkoetter U.
    J Lipid Res; 2009 Aug 15; 50(8):1556-62. PubMed ID: 18980943
    [Abstract] [Full Text] [Related]

  • 10. Gestational, pathologic and biochemical differences between very long-chain acyl-CoA dehydrogenase deficiency and long-chain acyl-CoA dehydrogenase deficiency in the mouse.
    Cox KB, Hamm DA, Millington DS, Matern D, Vockley J, Rinaldo P, Pinkert CA, Rhead WJ, Lindsey JR, Wood PA.
    Hum Mol Genet; 2001 Sep 15; 10(19):2069-77. PubMed ID: 11590124
    [Abstract] [Full Text] [Related]

  • 11. A heterozygous missense mutation in adolescent-onset very long-chain acyl-CoA dehydrogenase deficiency with exercise-induced rhabdomyolysis.
    Hisahara S, Matsushita T, Furuyama H, Tajima G, Shigematsu Y, Imai T, Shimohama S.
    Tohoku J Exp Med; 2015 Apr 15; 235(4):305-10. PubMed ID: 25843429
    [Abstract] [Full Text] [Related]

  • 12. Atypical presentation of VLCAD deficiency associated with a novel ACADVL splicing mutation.
    Shchelochkov O, Wong LJ, Shaibani A, Shinawi M.
    Muscle Nerve; 2009 Mar 15; 39(3):374-82. PubMed ID: 19208414
    [Abstract] [Full Text] [Related]

  • 13. Diagnostic assessment and long-term follow-up of 13 patients with Very Long-Chain Acyl-Coenzyme A dehydrogenase (VLCAD) deficiency.
    Laforêt P, Acquaviva-Bourdain C, Rigal O, Brivet M, Penisson-Besnier I, Chabrol B, Chaigne D, Boespflug-Tanguy O, Laroche C, Bedat-Millet AL, Behin A, Delevaux I, Lombès A, Andresen BS, Eymard B, Vianey-Saban C.
    Neuromuscul Disord; 2009 May 15; 19(5):324-9. PubMed ID: 19327992
    [Abstract] [Full Text] [Related]

  • 14. Effect of heat stress and bezafibrate on mitochondrial beta-oxidation: comparison between cultured cells from normal and mitochondrial fatty acid oxidation disorder children using in vitro probe acylcarnitine profiling assay.
    Li H, Fukuda S, Hasegawa Y, Kobayashi H, Purevsuren J, Mushimoto Y, Yamaguchi S.
    Brain Dev; 2010 May 15; 32(5):362-70. PubMed ID: 19589653
    [Abstract] [Full Text] [Related]

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  • 16. Concerted action of leptin in regulation of fatty acid oxidation in skeletal muscle and liver.
    Wein S, Ukropec J, Gasperíková D, Klimes I, Seböková E.
    Exp Clin Endocrinol Diabetes; 2007 Apr 15; 115(4):244-51. PubMed ID: 17479441
    [Abstract] [Full Text] [Related]

  • 17. Novel mutation of early, perinatal-onset, myopathic-type very-long-chain acyl-CoA dehydrogenase deficiency.
    Korematsu S, Kosugi Y, Kumamoto T, Yamaguchi S, Izumi T.
    Pediatr Neurol; 2009 Aug 15; 41(2):151-3. PubMed ID: 19589468
    [Abstract] [Full Text] [Related]

  • 18. De novo fatty acid biosynthesis and elongation in very long-chain acyl-CoA dehydrogenase-deficient mice supplemented with odd or even medium-chain fatty acids.
    Tucci S, Behringer S, Spiekerkoetter U.
    FEBS J; 2015 Nov 15; 282(21):4242-53. PubMed ID: 26284828
    [Abstract] [Full Text] [Related]

  • 19. Very long chain acyl-coenzyme A dehydrogenase deficiency with adult onset.
    Smelt AH, Poorthuis BJ, Onkenhout W, Scholte HR, Andresen BS, van Duinen SG, Gregersen N, Wintzen AR.
    Ann Neurol; 1998 Apr 15; 43(4):540-4. PubMed ID: 9546340
    [Abstract] [Full Text] [Related]

  • 20. Tissue-specific strategies of the very-long chain acyl-CoA dehydrogenase-deficient (VLCAD-/-) mouse to compensate a defective fatty acid β-oxidation.
    Tucci S, Herebian D, Sturm M, Seibt A, Spiekerkoetter U.
    PLoS One; 2012 Apr 15; 7(9):e45429. PubMed ID: 23024820
    [Abstract] [Full Text] [Related]

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