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Papers In Press, published online ahead of print April 1, 2005
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Laboratory Genetic Metabolic Disease, Academic Medical Center, Amsterdam 1105 AZ
Corresponding Author: f.m.vaz{at}amc.nl
Barth syndrome is an X-linked recessive disorder, which is biochemically characterized by low cellular levels of the mitochondrial phospholipid cardiolipin. Previously, we discovered that the yeast disruptant of the TAZ orthologue in Saccharomyces cerevisiae not only displays cardiolipin deficiency but also accumulates monolysocardiolipins, which are intermediates in cardiolipin remodeling. We therefore set out to investigate whether monolysocardiolipin accumulation also occurs in Barth syndrome. Indeed, we observed monolysocardiolipin accumulation in heart, muscle, lymphocytes and cultured lymphoblasts of Barth syndrome 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 monolysocardiolipins was different depending on the tissue source, it did parallel the fatty acid composition of the (remaining) cardiolipins. The possible implications of these findings for the two reported cardiolipin remodeling mechanisms, transacylation and deacylation/reacylation, are discussed. Because monolysocardiolipins have been proposed to be involved in the initiation of apoptosome-mediated cell-death by the sequestration of the pro-apoptotic protein (t)Bid to the mitochondrial membrane, we used control and Barth syndrome lymphoblasts to investigate whether the accumulation of monolysocardiolipins 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 monolysocardiolipin accumulation does not lead to enhanced apoptosis in cultured Barth syndrome lymphoblasts.
Revised on March 23, 2005
Accepted on March 23, 2005
Monolysocardiolipins accumulate in Barth syndrome but do not lead to enhanced apoptosis
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