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The TIM23 mitochondrial protein import complex: function and dysfunction

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Abstract

Mitochondria acquire the majority of their proteins from the cytosol in a process that is mediated by intricate multimeric machineries designed to allow proteins to cross and/or to insert themselves into the two mitochondrial membranes. Ongoing studies carried out in yeast over the past few decades have led to the discovery of numerous protein components that constitute several mitochondrial translocases. One of these complexes, the mitochondrial TIM23, is the major translocase for matrix proteins and is the focus of this review. The components of the TIM23 complex are categorized into four functional types. The first type plays the role of receptor for preproteins in the intermembrane space. The second type forms the actual channel that allows proteins to cross the inner mitochondrial membrane. The third species functions as part of the motor that mediates the final steps of import across the inner membrane. Additional components play regulatory roles orchestrating the action of this myriad of subunits. Recent studies provide new insights into the function of the mammalian TIM23 complex and the role that it plays under pathological conditions.

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Acknowledgments

We thank Dana Dayan and Dr. Celeste Weiss for useful comments and discussion.

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Authors and Affiliations

  1. Department of Biochemistry and Molecular Biology, The George S. Wise Faculty of Life Sciences, Tel Aviv University, Tel Aviv, 69978, Israel

    Keren Demishtein-Zohary & Abdussalam Azem

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  1. Keren Demishtein-Zohary
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  2. Abdussalam Azem
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Corresponding author

Correspondence to Abdussalam Azem.

Additional information

A.A. is supported by the Israel Science Foundation (ISF-1507/13) and the DFG trilateral project (Reference number SCHO 754/5-2).

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Demishtein-Zohary, K., Azem, A. The TIM23 mitochondrial protein import complex: function and dysfunction. Cell Tissue Res 367, 33–41 (2017). https://doi.org/10.1007/s00441-016-2486-7

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  • DOI: https://doi.org/10.1007/s00441-016-2486-7

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