Gene Symbol: TIM13
Description: protein translocase subunit TIM13
Alias: protein translocase subunit TIM13
Species: Saccharomyces cerevisiae S288c

Top Publications

  1. Koehler C, Leuenberger D, Merchant S, Renold A, Junne T, Schatz G. Human deafness dystonia syndrome is a mitochondrial disease. Proc Natl Acad Sci U S A. 1999;96:2141-6 pubmed
    ..The deafness dystonia syndrome thus is a novel type of mitochondrial disease that probably is caused by a defective mitochondrial protein-import system...
  2. Kurz M, Martin H, Rassow J, Pfanner N, Ryan M. Biogenesis of Tim proteins of the mitochondrial carrier import pathway: differential targeting mechanisms and crossing over with the main import pathway. Mol Biol Cell. 1999;10:2461-74 pubmed
  3. Davis A, Sepuri N, Holder J, Johnson A, Jensen R. Two intermembrane space TIM complexes interact with different domains of Tim23p during its import into mitochondria. J Cell Biol. 2000;150:1271-82 pubmed
    ..The positive charges are not required for interaction with the Tim9p-Tim10p complex, but are essential for cross-linking of Tim23p to components of the inner membrane insertion machinery, including Tim54p, Tim22p, and Tim12p. ..
  4. Müller J, Milenkovic D, Guiard B, Pfanner N, Chacinska A. Precursor oxidation by Mia40 and Erv1 promotes vectorial transport of proteins into the mitochondrial intermembrane space. Mol Biol Cell. 2008;19:226-36 pubmed
    ..Thus, oxidation driven by Mia40 and Erv1 determines vectorial transport of the precursors into the mitochondrial intermembrane space. ..
  5. Beverly K, Sawaya M, Schmid E, Koehler C. The Tim8-Tim13 complex has multiple substrate binding sites and binds cooperatively to Tim23. J Mol Biol. 2008;382:1144-56 pubmed publisher
    The Tim8-Tim13 complex, located in the mitochondrial intermembrane space, functions in the TIM22 import pathway that mediates the import of the mitochondrial carriers Tim23, Tim22, and Tim17 into the mitochondrial inner membrane...
  6. Terziyska N, Grumbt B, Kozany C, Hell K. Structural and functional roles of the conserved cysteine residues of the redox-regulated import receptor Mia40 in the intermembrane space of mitochondria. J Biol Chem. 2009;284:1353-63 pubmed publisher
    ..Furthermore, the disulfide bond in the CPC segment mediates the redox reactions with the thiol oxidase Erv1 and substrate proteins in mitochondria. ..
  7. Curran S, Leuenberger D, Schmidt E, Koehler C. The role of the Tim8p-Tim13p complex in a conserved import pathway for mitochondrial polytopic inner membrane proteins. J Cell Biol. 2002;158:1017-27 pubmed
    ..Thus, polytopic inner membrane proteins, which lack an NH2-terminal targeting sequence, pass through the TOM complex as a loop followed by binding of the small Tim proteins to the hydrophobic membrane spanning domains. ..
  8. Mesecke N, Terziyska N, Kozany C, Baumann F, Neupert W, Hell K, et al. A disulfide relay system in the intermembrane space of mitochondria that mediates protein import. Cell. 2005;121:1059-69 pubmed
    ..We suggest that this process reflects the evolutionary origin of the IMS from the periplasmic space of the prokaryotic ancestors of mitochondria. ..
  9. Tienson H, Dabir D, Neal S, Loo R, Hasson S, Boontheung P, et al. Reconstitution of the mia40-erv1 oxidative folding pathway for the small tim proteins. Mol Biol Cell. 2009;20:3481-90 pubmed publisher
    ..Here, we have reconstituted the oxidative folding pathway in vitro with Tim13 as a substrate and determined the midpoint potentials of Mia40 and Tim13...

More Information


  1. Rampello A, Glynn S. Identification of a Degradation Signal Sequence within Substrates of the Mitochondrial i-AAA Protease. J Mol Biol. 2017;429:873-885 pubmed publisher
    ..Together, these results identify the first specific degron sequence within a native i-AAA protease substrate. ..
  2. Leuenberger D, Bally N, Schatz G, Koehler C. Different import pathways through the mitochondrial intermembrane space for inner membrane proteins. EMBO J. 1999;18:4816-22 pubmed
    ..Yeast mitochondria thus use multiple pathways for escorting hydrophobic inner membrane proteins across the aqueous intermembrane space. ..
  3. Curran S, Leuenberger D, Leverich E, Hwang D, Beverly K, Koehler C. The role of Hot13p and redox chemistry in the mitochondrial TIM22 import pathway. J Biol Chem. 2004;279:43744-51 pubmed
    ..Together these results suggest that the small TIM complexes have a specialized assembly pathway in the intermembrane space and that the local redox state of the TIM complexes may mediate translocation of inner membrane proteins. ..
  4. Terziyska N, Lutz T, Kozany C, Mokranjac D, Mesecke N, Neupert W, et al. Mia40, a novel factor for protein import into the intermembrane space of mitochondria is able to bind metal ions. FEBS Lett. 2005;579:179-84 pubmed
    ..Studies on one of these proteins, Tim13, revealed that net translocation across the outer membrane is driven by metal-dependent folding in the IMS ...