Identification and characterization of human cardiolipin synthase |
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Authors: | Houtkooper Riekelt H Akbari Hana van Lenthe Henk Kulik Willem Wanders Ronald J A Frentzen Margrit Vaz Frédéric M |
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Institution: | Department of Clinical Chemistry, Laboratory Genetic Metabolic Diseases, University of Amsterdam, Academic Medical Center, P.O. Box 22700, 1100 DE Amsterdam, The Netherlands. |
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Abstract: | The mitochondrial phospholipid cardiolipin is synthesized from cytidinediphosphate-diacylglycerol and phosphatidylglycerol, a process catalyzed by the enzyme cardiolipin synthase. In this study, we identified a human candidate gene/cDNA for cardiolipin synthase, C20orf155. Expression of this candidate cDNA in the (cardiolipin synthase-deficient) crd1Delta yeast confirmed that it indeed encodes human cardiolipin synthase. Purified mitochondria of the crd1Delta expressing human cardiolipin synthase were used to characterize the enzyme. It has an alkaline pH optimum, requires divalent cations for activity and appears to have a different substrate preference for cytidinediphosphate-diacylglycerol species when compared to phosphatidylglycerol species. The possible implications for CL synthesis and remodeling are discussed. |
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Keywords: | At Arabidopsis thaliana BTP Bis-Tris propane CDP-DAG cytidinediphosphate-diacylglycerol CL cardiolipin CLS cardiolipin synthase Hs Homo sapiens PG phosphatidylglycerol Sc Saccharomyces cerevisiae |
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