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Journal of Lipid Research, Vol. 46, 1182-1195, June 2005
Copyright © 2005 by American Society for Biochemistry and Molecular Biology
* Laboratory of Genetic Metabolic Diseases, Departments of Clinical Chemistry and Pediatrics, Emma Children's Hospital, Academic Medical Center, University of Amsterdam, 1100 DE Amsterdam, The Netherlands
Murdoch Childrens Research Institute, Royal Children's Hospital and Department of Paediatrics, University of Melbourne, Melbourne, Victoria 3052, Australia
Hopital Necker Enfants Malades, Department of Genetics and Institut National de la Santé et de la Recherche Médicale U-393, 75743 Paris, Cedex 15, France
** Baylor College of Medicine and Texas Children's Hospital, Department of Pediatrics (Cardiology), Houston, TX
Walter and Eliza Hall Institute of Medical Research, the Murdoch Childrens Research Institute, and Department of Neonatology, Royal Children's Hospital, Melbourne, Victoria 3052, Australia
Published, JLR Papers in Press, April 1, 2005. DOI 10.1194/jlr.M500056-JLR200
1 To whom correspondence should be addressed. e-mail: f.m.vaz{at}amc.uva.nl
Barth syndrome (BTHS) is an X-linked recessive disorder that is biochemically characterized by low cellular levels of the mitochondrial phospholipid cardiolipin (CL). Previously, we discovered that the yeast disruptant of the TAZ ortholog in Saccharomyces cerevisiae not only displays CL deficiency but also accumulates monolysocardiolipins (MLCLs), which are intermediates in CL remodeling. Therefore, we set out to investigate whether MLCL accumulation also occurs in BTHS. Indeed, we observed MLCL accumulation in heart, muscle, lymphocytes, and cultured lymphoblasts of BTHS patients; however, only very low levels of these lysophospholipids were found in platelets and fibroblasts of these patients. Although the fatty acid composition of the MLCLs was different depending on the tissue source, it did parallel the fatty acid composition of the (remaining) CLs. The possible implications of these findings for the two reported CL remodeling mechanisms, transacylation and deacylation/reacylation, are discussed. Because MLCLs have been proposed to be involved in the initiation of apoptosome-mediated cell death by the sequestration of the proapoptotic protein (t)BH3-interacting domain death agonist (Bid) to the mitochondrial membrane, we used control and BTHS lymphoblasts to investigate whether the accumulation of MLCLs results in higher levels of apoptosis.
We found no differences in susceptibility to death receptor-mediated apoptosis or in cellular distribution of Bid, cytochrome c, and other parameters, implying that MLCL accumulation does not lead to enhanced apoptosis in cultured BTHS lymphoblasts.
Abbreviations: Bid, BH3-interacting domain death agonist; BTHS, Barth syndrome; CL, cardiolipin; FasL, Fas ligand; I.S., internal standard; MLCL, monolysocardiolipin; PARP, poly (ADP-ribose) polymerase; PC, phosphatidylcholine; PE, phosphatidylethanolamine
Supplementary key words cardiolipin • tafazzin • lymphoblasts
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