Experts and Doctors on long chain acyl coa dehydrogenase in Düsseldorf, North Rhine Westphalia, Germany
Locale: Düsseldorf, North Rhine Westphalia, Germany
Topic: long chain acyl coa dehydrogenase
Spiekerkoetter U, Tokunaga C, Wendel U, Mayatepek E, Exil V, Duran M, et al
. Changes in blood carnitine and acylcarnitine profiles of very long-chain acyl-CoA dehydrogenase-deficient mice subjected to stress. Eur J Clin Invest. 2004;34:191-6 pubmed
..Monitoring blood acylcarnitine profiles in response to different stressors may allow systematic analysis of therapeutic interventions in VLCAD knockout mice. ..
Spiekerkoetter U, Tokunaga C, Wendel U, Mayatepek E, IJlst L, Vaz F, et al
. Tissue carnitine homeostasis in very-long-chain acyl-CoA dehydrogenase-deficient mice. Pediatr Res. 2005;57:760-4 pubmed
..Our data suggest that carnitine supplementation in long-chain beta-oxidation defects may not be required, and blood carnitine concentrations do not reflect tissue carnitine homeostasis. ..
Spiekerkoetter U, Ruiter J, Tokunaga C, Wendel U, Mayatepek E, Wijburg F, et al
. Evidence for impaired gluconeogenesis in very long-chain acyl-CoA dehydrogenase-deficient mice. Horm Metab Res. 2006;38:625-30 pubmed
..Whether this is due to lack of a substrate, inhibitory effects on other gluconeogenic enzymes or impaired transcription of gluconeogenic enzymes needs to be resolved in the future. ..
ter Veld F, Primassin S, Hoffmann L, Mayatepek E, Spiekerkoetter U. Corresponding increase in long-chain acyl-CoA and acylcarnitine after exercise in muscle from VLCAD mice. J Lipid Res. 2009;50:1556-62 pubmed publisher
..This is the first study demonstrating that acylcarnitines and acyl-CoA directly correlate and concomitantly increase after exercise in VLCAD-deficient muscle. ..
ter Veld F, Mueller M, Kramer S, Haussmann U, Herebian D, Mayatepek E, et al
. A novel tandem mass spectrometry method for rapid confirmation of medium- and very long-chain acyl-CoA dehydrogenase deficiency in newborns. PLoS ONE. 2009;4:e6449 pubmed publisher
..Importantly, based on measured MCAD residual activities in correlation with genotype, new insights were obtained on the expected clinical phenotype. ..