Transport into mitochondria and intramitochondrial sorting of the Fe/S protein of ubiquinol-cytochrome c reductase |
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| Authors: | F U Hartl B Schmidt E Wachter H Weiss W Neupert |
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| Institution: | 1. Department of Clinical Laboratory, The 3rd People''s Hospital of Qingdao, Qingdao 266041, Shandong Province, China;2. Department of Clinical Laboratory, Qingdao Central Hospital, Qingdao 266042, Shandong Province, China;1. Department of Biochemistry, Stanford University, 94305, Stanford, CA, USA;2. Centre for Neuroscience and Nanotechnology, Department of Biomedical, Metabolic and Neural Sciences, University of Modena and Reggio, Emilia, Modena, Italy;3. Department of Pathology, Stanford University, 94305, Stanford, CA, USA;4. Biotechnology Center (BIOTEC), Center for Molecular and Cellular Bioengineering (CMCB), Technische Universitat Dresden, Tatzberg 47/49, 01307, Dresden, Germany;5. Department of Biochemistry and Molecular Biophysics, Washington University School of Medicine, 63110, St. Louis, MO, USA;6. Center for Science and Engineering of Living Systems (CSELS), Washington University in St. Louis, 63130, St. Louis, MO, USA;1. From the Department of Biological Sciences, Purdue University, 915 W. State St., LILY G-227, 47907, West Lafayette, IN, USA;2. Department of Computer Science, Purdue University, 47907, West Lafayette, IN, USA;3. Bindley Biosciences Center, Purdue University, 47907, West Lafayette, IN, USA;1. Institute of Molecular and Cell Biology, Agency for Science, Technology, and Research (A*STAR), 61 Biopolis Drive, Proteos, Singapore 138673, Singapore;2. School of Biological Sciences, Nanyang Technological University, 60 Nanyang Drive, Singapore 637551, Singapore |
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| Abstract: | The Fe/S protein of complex III is encoded by a nuclear gene, synthesized in the cytoplasm as a precursor with a 32 residue amino-terminal extension, and transported to the outer surface of the inner mitochondrial membrane. Our data suggest the following transport pathway. First, the precursor is translocated via translocation contact sites into the matrix. There, cleavage to an intermediate containing an eight residue extension occurs. The intermediate is then redirected across the inner membrane, processed to the mature subunit, and assembled into complex III. We suggest that the folding and membrane-translocation pathway in the endosymbiotic ancestor of mitochondria has been conserved during evolution of eukaryotic cells; transfer of the gene for Fe/S protein to the nucleus has led to addition of the presequence, which routes the precursor back to its "ancestral" assembly pathway. |
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