Journal article
Mitochondrial Cardiolipin Involved in Outer-Membrane Protein Biogenesis: Implications for Barth Syndrome
N Gebert, AS Joshi, S Kutik, T Becker, M McKenzie, XL Guan, VP Mooga, DA Stroud, G Kulkarni, MR Wenk, P Rehling, C Meisinger, MT Ryan, N Wiedemann, ML Greenberg, N Pfanner
Current Biology | CELL PRESS | Published : 2009
Abstract
The biogenesis of mitochondria requires the import of a large number of proteins from the cytosol [1, 2]. Although numerous studies have defined the proteinaceous machineries that mediate mitochondrial protein sorting, little is known about the role of lipids in mitochondrial protein import. Cardiolipin, the signature phospholipid of the mitochondrial inner membrane [3-5], affects the stability of many inner-membrane protein complexes [6-12]. Perturbation of cardiolipin metabolism leads to the X-linked cardioskeletal myopathy Barth syndrome [13-18]. We report that cardiolipin affects the preprotein translocases of the mitochondrial outer membrane. Cardiolipin mutants genetically interact wit..
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Awarded by National Institutes of Health
Funding Acknowledgements
We thank B. Schonfisch for expert technical assistance. This work was supported by the Deutsche Forschungsgemeinschaft (P.R., C.M., and N.P.), Sonderforschungsbereich 746, the Excellence Initiative of the German federal and state governments (EXC 294), the Gottfried Wilhelm Leibniz Program, Landesforschungspreis Baden-WCirttemberg, Fonds der Chemischen Industrie (N.P.), grant HLO84218 from the National Institutes of Health (M.L.G.), the Barth Syndrome Foundation (M.L.G.), the Australian Research Council, the National Health and Medical Research Council of Australia (M.T.R.), Singapore National Research Foundation Competitive Research Program award 2007-04 (M.R.W.), Landesstiftung Baden-WUrttemberg (T.B.), Trinational Research Training Group GRK 1478 (C.M., fellowship to D.A.S.), and a Boehringer Ingelheirn Fonds predoctoral fellowship (S.K.).