Gene Symbol: TIMM10
Description: translocase of inner mitochondrial membrane 10
Alias: TIM10, TIM10A, TIMM10A, mitochondrial import inner membrane translocase subunit Tim10, translocase of inner mitochondrial membrane 10 homolog
Species: human
Products:     TIMM10

Top Publications

  1. Baker M, Webb C, Stroud D, Palmer C, Frazier A, Guiard B, et al. Structural and functional requirements for activity of the Tim9-Tim10 complex in mitochondrial protein import. Mol Biol Cell. 2009;20:769-79 pubmed publisher
    The Tim9-Tim10 complex plays an essential role in mitochondrial protein import by chaperoning select hydrophobic precursor proteins across the intermembrane space...
  2. Smith J, Singha U, Misra S, Chaudhuri M. Divergent Small Tim Homologues Are Associated with TbTim17 and Critical for the Biogenesis of TbTim17 Protein Complexes in Trypanosoma brucei. mSphere. 2018;3: pubmed publisher
    ..brucei Tim9 (TbTim9) and Tim10 (TbTim10) and a unique small Tim that shares homology with both Tim8 and Tim13 (TbTim8/13)...
  3. Milenkovic D, Ramming T, Müller J, Wenz L, Gebert N, Schulze Specking A, et al. Identification of the signal directing Tim9 and Tim10 into the intermembrane space of mitochondria. Mol Biol Cell. 2009;20:2530-9 pubmed publisher
    ..In this study, we found that a region within the Tim9 and Tim10 precursors, consisting of only nine amino acid residues, functions as a signal for the engagement of substrate ..
  4. Spiller M, Guo L, Wang Q, Tran P, Lu H. Mitochondrial Tim9 protects Tim10 from degradation by the protease Yme1. Biosci Rep. 2015;35: pubmed publisher
    Translocase of IM (inner membrane; Tim)9 and Tim10 are essential homologue proteins of the mitochondrial intermembrane space (IMS) and form a stable hexameric Tim9-Tim10 complex there...
  5. Sambarey A, Devaprasad A, Mohan A, Ahmed A, Nayak S, Swaminathan S, et al. Unbiased Identification of Blood-based Biomarkers for Pulmonary Tuberculosis by Modeling and Mining Molecular Interaction Networks. EBioMedicine. 2017;15:112-126 pubmed publisher
    ..on independent patient samples, and identify a signature comprising 10 genes - FCGR1A, HK3, RAB13, RBBP8, IFI44L, TIMM10, BCL6, SMARCD3, CYP4F3 and SLPI, that can discriminate between TB and healthy controls as well as distinguish TB ..
  6. 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
    ..i-AAA protease, Yme1p, to examine the in vitro degradation of two intermembrane space chaperone subunits, Tim9 and Tim10. Yme1p degrades Tim10 more rapidly than Tim9 despite high sequence and structural similarity, and loss of Tim10 is ..
  7. Bauer M, Rothbauer U, Mühlenbein N, Smith R, Gerbitz K, Neupert W, et al. The mitochondrial TIM22 preprotein translocase is highly conserved throughout the eukaryotic kingdom. FEBS Lett. 1999;464:41-7 pubmed
    ..DDP1 shows similarity to the yeast proteins Tim9, Tim10 and Tim12, components of the mitochondrial import machinery for carrier proteins...
  8. Neal S, Dabir D, Wijaya J, Boon C, Koehler C. Osm1 facilitates the transfer of electrons from Erv1 to fumarate in the redox-regulated import pathway in the mitochondrial intermembrane space. Mol Biol Cell. 2017;28:2773-2785 pubmed publisher
    ..From in vitro import assays, mitochondria lacking Osm1 display decreased import of MIA substrates, Cmc1 and Tim10. Comparative reconstitution assays support that the Osm1/fumarate couple accepts electrons with similar efficiency ..
  9. Koehler C, Jarosch E, Tokatlidis K, Schmid K, Schweyen R, Schatz G. Import of mitochondrial carriers mediated by essential proteins of the intermembrane space. Science. 1998;279:369-73 pubmed
    ..Neither protein was required for protein import into the other mitochondrial compartments. Both proteins may function as intermembrane space chaperones for the highly insoluble carrier proteins. ..

More Information


  1. Vial S, Lu H, Allen S, Savory P, Thornton D, Sheehan J, et al. Assembly of Tim9 and Tim10 into a functional chaperone. J Biol Chem. 2002;277:36100-8 pubmed
    The TIM10 complex is localized in the mitochondrial intermembrane space and mediates insertion of hydrophobic proteins at the inner membrane...
  2. Mühlenbein N, Hofmann S, Rothbauer U, Bauer M. Organization and function of the small Tim complexes acting along the import pathway of metabolite carriers into mammalian mitochondria. J Biol Chem. 2004;279:13540-6 pubmed
    Tim9, Tim10a, and Tim10b are members of the family of small Tim proteins located in the intermembrane space of mammalian mitochondria...
  3. Webb C, Gorman M, Lazarou M, Ryan M, Gulbis J. Crystal structure of the mitochondrial chaperone TIM9.10 reveals a six-bladed alpha-propeller. Mol Cell. 2006;21:123-33 pubmed
    ..Tim9 and Tim10 are translocase components of the intermembrane space, related to deafness-dystonia peptide 1 (DDP1)...
  4. Jin H, Kendall E, Freeman T, Roberts R, Vetrie D. The human family of Deafness/Dystonia peptide (DDP) related mitochondrial import proteins. Genomics. 1999;61:259-67 pubmed
    ..We also describe the expression patterns and chromosomal locations of their genes, which are candidate loci for autosomal recessive neurodegenerative disorders. ..
  5. Rothbauer U, Hofmann S, Mühlenbein N, Paschen S, Gerbitz K, Neupert W, et al. Role of the deafness dystonia peptide 1 (DDP1) in import of human Tim23 into the inner membrane of mitochondria. J Biol Chem. 2001;276:37327-34 pubmed
    ..Thus, the pathomechanism underlying the Mohr-Tranebjaerg syndrome may involve an impaired biogenesis of the human TIM23 complex causing severe pleiotropic mitochondrial dysfunction. ..
  6. Carey K, Segal D, Klein R, Sanigorski A, Walder K, Collier G, et al. Identification of novel genes expressed during rhabdomyosarcoma differentiation using cDNA microarrays. Pathol Int. 2006;56:246-55 pubmed
    ..Prothymosin alpha (PTMA), and translocase of inner mitochondrial membrane 10 (Tim10), two genes not previously implicated in RMS, showed reduced expression during differentiation...
  7. Allen S, Lu H, Thornton D, Tokatlidis K. Juxtaposition of the two distal CX3C motifs via intrachain disulfide bonding is essential for the folding of Tim10. J Biol Chem. 2003;278:38505-13 pubmed
    The TIM10 complex, composed of the homologous proteins Tim10 and Tim9, chaperones hydrophobic proteins inserted at the mitochondrial inner membrane...