Mitochondrial Cardiolipin Involved in Outer-Membrane Protein Biogenesis: Implications for Barth Syndrome

作者：Gebert Natalia; Joshi Amit S; Kutik Stephan; Becker Thomas; McKenzie Matthew; Guan Xue Li; Mooga Ved P; Stroud David A; Kulkarni Gnanada; Wenk Markus R; Rehling Peter; Meisinger Chris; Ryan Michael T; Wiedemann Nils; Greenberg Miriam L; Pfanner Nikolaus^*

来源：Current Biology, 2009, 19(24): 2133-2139.

DOI：10.1016/j.cub.2009.10.074

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 cardio-skeletal myopathy Barth syndrome [13-18]. We report that cardiolipin affects the preprotein translocases of the mitochondrial outer membrane. Cardiolipin mutants genetically interact with mutants of outer-membrane translocases. Mitochondria from cardiolipin yeast mutants, as well as Barth syndrome patients, are impaired in the biogenesis of outer-membrane proteins. Our findings reveal a new role for cardiolipin in protein sorting at the mitochondrial outer membrane and bear implications for the pathogenesis of Barth syndrome.

• 出版日期2009-12-29