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Science 16 January 1998:
Vol. 279. no. 5349, pp. 369 - 373
DOI: 10.1126/science.279.5349.369
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Reports

Import of Mitochondrial Carriers Mediated by Essential Proteins of the Intermembrane Space

Carla M. Koehler, Ernst Jarosch, Kostas Tokatlidis, Karl Schmid, Rudolf J. Schweyen, Gottfried Schatz *

In order to reach the inner membrane of the mitochondrion, multispanning carrier proteins must cross the aqueous intermembrane space. Two essential proteins of that space, Tim10p and Tim12p, were shown to mediate import of multispanning carriers into the inner membrane. Both proteins formed a complex with the inner membrane protein Tim22p. Tim10p readily dissociated from the complex and was required to transport carrier precursors across the outer membrane; Tim12p was firmly bound to Tim22p and mediated the insertion of carriers into the inner membrane. Neither protein was required for protein import into the other mitochondrial compartments. Both proteins may function as intermembrane space chaperones for the highly insoluble carrier proteins.

C. M. Koehler, K. Tokatlidis, K. Schmid, G. Schatz, Biozentrum, University of Basel, Klingelbergstrasse 70, CH-4056 Basel, Switzerland.
E. Jarosch and R. J. Schweyen, Institut für Mikrobiologie und Genetik, University of Vienna, A-1030 Vienna, Austria.
*   To whom correspondence should be addressed. E-mail: schatz{at}ubaclu.unibas.ch

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D. C. Wallace and D. G. Murdock (1999)
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Human deafness dystonia syndrome is a mitochondrial disease.
C. M. Koehler, D. Leuenberger, S. Merchant, A. Renold, T. Junne, and G. Schatz (1999)
PNAS 96, 2141-2146
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Tim23p Contains Separate and Distinct Signals for Targeting to Mitochondria and Insertion into the Inner Membrane.
A. J. Davis, K. R. Ryan, and R. E. Jensen (1998)
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   Abstract »    Full Text »    PDF »
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K. Saeki, H. Suzuki, M. Tsuneoka, M. Maeda, R. Iwamoto, H. Hasuwa, S. Shida, T. Takahashi, M. Sakaguchi, T. Endo, et al. (2000)
J. Biol. Chem. 275, 31996-32002
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Modular Structure of the TIM23 Preprotein Translocase of Mitochondria.
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J. Biol. Chem. 276, 25856-25861
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U. Rothbauer, S. Hofmann, N. Muhlenbein, S. A. Paschen, K.-D. Gerbitz, W. Neupert, M. Brunner, and M. F. Bauer (2001)
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