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Phospholipid abnormalities in children with Barth syndrome

Michael Schlame, MD^*,*, Richard I. Kelley, MD, PhD, Annette Feigenbaum, MB, ChB, Jeffrey A. Towbin, MD, Paul M. Heerdt, MD, PhD^||, Thomas Schieble, MD^*, Ronald J. A. Wanders, PhD^¶, Salvatore DiMauro, MD^# and Thomas J. J. Blanck, MD, PhD^*

^* Department of Anesthesiology, New York University School of Medicine, New York, New York, USA
Division of Metabolism, Kennedy Krieger Institute, Baltimore, Maryland, USA
Division of Clinical and Metabolic Genetics, The Hospital for Sick Children, Toronto, Canada
Department of Pediatrics (Cardiology), Baylor College of Medicine, Houston, Texas, USA
^|| Department of Anesthesiology, Weill Medical College of Cornell University, New York, New York, USA
^¶ Laboratory of Genetic Metabolic Diseases, Academic Medical Center, Amsterdam, The Netherlands
^# Department of Neurology, College of Physicians and Surgeons, Columbia University, New York, New York, USA

View larger version (27K): [in a new window]   Figure 1 Molecular composition of cardiac phosphatidylcholine (PC) and phosphatidylethanolamine (PE) from controls and Barth syndrome (BTHS) patients with tafazzin (TAZ) mutation. Data are means of four measurements (two left ventricle samples and two right ventricle samples). Asterisks indicate a statistically significant difference between control and BTHS (p < 0.05). Diacylglycerol species are listed in the sequence in which they elute from the chromatography column. 16:0 = palmitic acid; 18:0 = stearic acid; 18:1 = oleic acid; 18:2 = linoleic acid; 20:4 = arachidonic acid; 22:5 = docosapentaenoic acid; 22:6 = docosahexaenoic acid.