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Gene Review

TIM8  -  protein transporter TIM8

Saccharomyces cerevisiae S288c

Synonyms: Mitochondrial import inner membrane translocase subunit TIM8, YJR135BW, YJR135W-A
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Disease relevance of TIM8


High impact information on TIM8

  • The nonessential Tim8-Tim13 complex of the intermembrane space is not involved in the transfer of AAC across the outer membrane [2].
  • The TIM22 protein import pathway of the yeast mitochondrion contains several components, including a family of five proteins (Tim8p, -9p, -10p, -12p, and -13p [Tim, for translocase of inner membrane]) that are located in the intermembrane space and are 25% identical [3].
  • Membrane insertion involves binding of newly imported Phb1 to Tim8/13 complexes in the intermembrane space and is mediated by the TIM23-translocase [4].
  • Thus, our data suggest a model in which one of the first steps in Tom40 assembly may be interaction with the Tim8-Tim13 complex [5].
  • The Tim8-Tim13 complex of Neurospora crassa functions in the assembly of proteins into both mitochondrial membranes [5].

Biological context of TIM8


Anatomical context of TIM8

  • The demonstration of mitochondrial abnormalities in skeletal muscle biopsies in some patients is compatible with the suggestion from recent research that the TIMM8a protein is the human counterpart of an intermembrane mitochondrial transport protein, Tim8p, recently characterized in yeast [6].


  1. The role of the TIM8-13 complex in the import of Tim23 into mitochondria. Paschen, S.A., Rothbauer, U., Káldi, K., Bauer, M.F., Neupert, W., Brunner, M. EMBO J. (2000) [Pubmed]
  2. Mitochondrial import of the ADP/ATP carrier: the essential TIM complex of the intermembrane space is required for precursor release from the TOM complex. Truscott, K.N., Wiedemann, N., Rehling, P., Müller, H., Meisinger, C., Pfanner, N., Guiard, B. Mol. Cell. Biol. (2002) [Pubmed]
  3. The essential function of the small Tim proteins in the TIM22 import pathway does not depend on formation of the soluble 70-kilodalton complex. Murphy, M.P., Leuenberger, D., Curran, S.P., Oppliger, W., Koehler, C.M. Mol. Cell. Biol. (2001) [Pubmed]
  4. Formation of membrane-bound ring complexes by prohibitins in mitochondria. Tatsuta, T., Model, K., Langer, T. Mol. Biol. Cell (2005) [Pubmed]
  5. The Tim8-Tim13 complex of Neurospora crassa functions in the assembly of proteins into both mitochondrial membranes. Hoppins, S.C., Nargang, F.E. J. Biol. Chem. (2004) [Pubmed]
  6. Neuronal cell death in the visual cortex is a prominent feature of the X-linked recessive mitochondrial deafness-dystonia syndrome caused by mutations in the TIMM8a gene. Tranebjaerg, L., Jensen, P.K., Van Ghelue, M., Vnencak-Jones, C.L., Sund, S., Elgjo, K., Jakobsen, J., Lindal, S., Warburg, M., Fuglsang-Frederiksen, A., Skullerud, K. Ophthalmic Genet. (2001) [Pubmed]
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