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Originally published In Press as doi:10.1074/jbc.M409194200 on September 21, 2004

J. Biol. Chem., Vol. 279, Issue 48, 50243-50249, November 26, 2004
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Zim17, a Novel Zinc Finger Protein Essential for Protein Import into Mitochondria*

Lena Burri{ddagger}, Katherine Vascotto{ddagger}, Steffen Fredersdorf§, Ralph Tiedt§, Michael N. Hall§, and Trevor Lithgow{ddagger}||

From the {ddagger}Russell Grimwade School of Biochemistry and Molecular Biology, University of Melbourne, Parkville 3010, Australia and §Biozentrum, University of Basel, CH-4056 Basel, Switzerland

Translocation of precursor proteins across the mitochondrial membranes requires the coordinated action of multisubunit translocases in the outer and inner membrane, and the driving force for translocation across the inner membrane is provided by the matrix-located heat shock protein 70 (mtHsp70). The central components of the protein import machinery are essential. Here we describe Zim17, an essential protein with a zinc finger motif involved in protein import into mitochondria. Comparative genomics suggested a correction to the open reading frame of YNL310c, the gene encoding Zim17 in Saccharomyces cerevisiae. The revised open reading frame codes for a classic mitochondrial targeting signal, which is processed from Zim17 in the mitochondrial matrix. Loss of Zim17 selectively diminishes import of proteins into the matrix of mitochondria, but this loss of Zim17 is partially suppressed by overexpression of the J-protein Pam18/Tim14. We propose that Zim17 functions as an example of a "fractured" J-protein, where a protein like Zim17 contributes a zinc finger domain to Type III J-proteins, in toto providing for substrate loading onto Hsp70.


Received for publication, August 11, 2004 , and in revised form, September 10, 2004.

* This work was supported by grants from the Australian Research Council (to T. L.) and a grant from the Australian Research Council (to L. B.), and support for a collaborative study leave was provided by the Human Frontiers Science Program (to T. L.). The costs of publication of this article were defrayed in part by the payment of page charges. This article must therefore be hereby marked "advertisement" in accordance with 18 U.S.C. Section 1734 solely to indicate this fact.

Current address: ICN Pharmaceuticals, CH-4056 Basel, Switzerland AG.

|| To whom correspondence should be addressed. Tel.: 61-3-8344-4131; Fax: 61-3-9348-2251; E-mail: t.lithgow{at}unimelb.edu.au.


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