Partenaires et organisations internationales
(Anglais)
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Centre for Biomembranes and Lipid Enzymology of the University, Utrecht (CH), Molecular Biology Laboratory Centre of Biotechnology, Huddinge (S), Department of Biochemistry, University of the Basque Country (E), Departimento de Scienze Biomediche Sperimentali of the University, Padova (I), Physiologische Chemie I, L.-M. Universität München (D), Institut Pasteur, Paris Cedex (F), Macromolecules at Interfaces Lab, Univ. libre de Bruxelles (B), EMBL, Heidelberg (D), Unidade de Ciencias Exactas e Humanas, Campus de Gambelas (P), Department of Biochemistry, Oxford (GB)
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Résumé des résultats (Abstract)
(Anglais)
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During this final part of the granting period, our laboratory has achieved the followîng:
1. In collaboration with one of our TMR partners, Dr. Ben de Kruijff at the University of Utrecht, we have shown that the ATP-driven protein import motor of the mitochondrial inner membrane is attached to that membrane via a lipid-protein interaction involving the essential protein Tim44p. This work is now in press in the Proc. Nat. Acad. Sci. USA.
2. We have defined the enzymic properties of the mitochondrial Lon protease and shown by high-resolution electron microscopy that the active enzyme forms a heptameric ring.
3. We have characterized a novel mitochondrial import system for polytopic inner membrane proteins and shown that mutation in this system can cause a serious deafness/dystonia/blindness syndrome in humans.
C. M. Koehler, E. Jarosch, K. Tokatlidis, K. Schmid, R. J. Schweyen and G. Schatz: Import of mitochondrial carriers mediated by essential proteins of the intermembrane space. Science 279, 369-373 (1998).
T. Komiya, S. Rospert, C. Koehler, R. Looser, G. Schatz and K. Mihara: Interaction of mitochondrial targeting signals with acidic receptor domains along the protein import pathway: evidence for the 'acid chain' hypothesis. EMBO J. 17, 3886-3898 (1998)
J. M. van Dijl, E. Kutejova, K. Suda, D. Pertecko, G. Schatz and C. K. Suzuki: The ATPase and protease domain of yeast mitochondrial Lon: Roles in proteolysis and respiration-dependent growth. Proc. Natl. Acad. Sci. USA 95, 10584-10589 (1998)
C. M. Koehler, S. Merchant, W. Oppliger, K. Schmid, E. Jarosch, L. Dolfini, T. Junne, G. Schatz and K. Tokatlidis: Tim9p, an essential partner for Tim10p for the import of mitochondrial carrier proteins. EMBO J. 17, 6477-6486 (1998)
C. M. Koehler, D. Leuenberger, S. Merchant, A. Renold and G. Schatz: Human deafness dystonia syndrome is a mitochondrial disease. Proc. Natl. Acad. Sci. USA 96, 2141-2146 (1999)
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