Universität zu Köln

Baker, Michael J., Mooga, Ved P., Guiard, Bernard, Langer, Thomas ORCID: 0000-0003-1250-1462, Ryan, Michael T. and Stojanovski, Diana ORCID: 0000-0002-0199-3222 (2012). Impaired Folding of the Mitochondria! Small TIM Chaperones Induces Clearance by the i-AAA Protease. J. Mol. Biol., 424 (5). S. 227 - 240. LONDON: ACADEMIC PRESS LTD- ELSEVIER SCIENCE LTD. ISSN 1089-8638

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Abstract

The intermembrane space of mitochondria contains a dedicated chaperone network-the small translocase of the inner membrane (TIM) family-for the sorting of hydrophobic precursors. All small TIMs are defined by the presence of a twin CX3C motif and the monomeric proteins are stabilized by two intramolecular disulfide bonds formed between the cysteines of these motifs. The conserved cysteine residues within small TIM members have also been shown to participate in early biogenesis events, with the most N-terminal cysteine residue important for import and retention within the intermembrane space via the receptor and disulfide oxidase, Mia40. In this study, we have analyzed the in vivo consequences of improper folding of small TIM chaperones by generating site-specific cysteine mutants and assessed the fate of the incompletely oxidized proteins within mitochondria. We show that no individual cysteine residue is required for the function of Tim9 or Tim10 in yeast and that defective assembly of the small TIMs induces their proteolytic clearance from mitochondria. We delineate a clearance mechanism for the mutant proteins and their unassembled wild-type partner protein by the mitochondrial ATP-dependent protease, Yme1 (yeast mitochondrial escape 1). (C) 2012 Elsevier Ltd. All rights reserved.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Baker, Michael J. UNSPECIFIED UNSPECIFIED UNSPECIFIED Mooga, Ved P. UNSPECIFIED UNSPECIFIED UNSPECIFIED Guiard, Bernard UNSPECIFIED UNSPECIFIED UNSPECIFIED Langer, Thomas UNSPECIFIED orcid.org/0000-0003-1250-1462 UNSPECIFIED Ryan, Michael T. UNSPECIFIED UNSPECIFIED UNSPECIFIED Stojanovski, Diana UNSPECIFIED orcid.org/0000-0002-0199-3222 UNSPECIFIED
URN:	urn:nbn:de:hbz:38-476716
DOI:	10.1016/j.jmb.2012.09.019
Journal or Publication Title:	J. Mol. Biol.
Volume:	424
Number:	5
Page Range:	S. 227 - 240
Date:	2012
Publisher:	ACADEMIC PRESS LTD- ELSEVIER SCIENCE LTD
Place of Publication:	LONDON
ISSN:	1089-8638
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language DISULFIDE RELAY SYSTEM; INTERMEMBRANE SPACE; TIM8-TIM13 COMPLEX; IMPORT CHANNEL; PROTEINS; MIA40; TRANSLOCATION; MEMBRANE; IDENTIFICATION; DEGRADATION Multiple languages Biochemistry & Molecular Biology Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/47671