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American Journal of Medical Genetics. Part A|April 21, 2004
X-linked cardioskeletal myopathy and neutropenia (Barth syndrome): an updatePeter G Barth, Fredoen Valianpour, Valerie M Bowen, et al.
Human Molecular Genetics|August 24, 2005
Bezafibrate increases very-long-chain acyl-CoA dehydrogenase protein and mRNA expression in deficient fibroblasts and is a potential therapy for fatty acid oxidation disordersF Djouadi, F Aubey, D Schlemmer, et al.
Hepatology (Baltimore, Md.)|March 30, 2007
Bile acid treatment alters hepatic disease and bile acid transport in peroxisome-deficient PEX2 Zellweger miceMegan H Keane, Henk Overmars, Thomas M Wikander, et al.
Biochemical and Biophysical Research Communications|August 22, 2006
First identification of a 2-ketoglutarate/isocitrate transport system in mammalian peroxisomes and its characterizationWouter F Visser, Carlo W T van Roermund, Lodewijk Ijlst, et al.
Hormone and Metabolic Research = Hormon- Und Stoffwechselforschung = Hormones Et Metabolisme|November 1, 2006
Evidence for impaired gluconeogenesis in very long-chain acyl-CoA dehydrogenase-deficient miceU Spiekerkoetter, J Ruiter, C Tokunaga, et al.
Cellular and Molecular Life Sciences : CMLS|October 31, 2007
Phytanic acid impairs mitochondrial respiration through protonophoric actionJ C Komen, F Distelmaier, W J H Koopman, et al.
Drug Metabolism and Disposition: the Biological Fate of Chemicals|October 16, 2004
Valproyl-dephosphoCoA: a novel metabolite of valproate formed in vitro in rat liver mitochondriaMargarida F B Silva, Lodewijk Ijlst, Paul Allers, et al.
European Journal of Clinical Investigation|January 11, 2005
Identification of fatty acid oxidation disorder patients with lowered acyl-CoA thioesterase activity in human skin fibroblastsM C Hunt, J Ruiter, P Mooyer, et al.
Molecular Genetics and Metabolism|September 2, 2003
Evidence for increased oxidative stress in peroxisomal D-bifunctional protein deficiencySacha Ferdinandusse, Barbara Finckh, Yvette C de Hingh, et al.
Molecular Genetics and Metabolism|January 27, 2005
Elongation of very long-chain fatty acids is enhanced in X-linked adrenoleukodystrophyStephan Kemp, Fredoen Valianpour, Simone Denis, et al.
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