mitochondrial proteins


Summary: Proteins encoded by the mitochondrial genome or proteins encoded by the nuclear genome that are imported to and resident in the MITOCHONDRIA.

Top Publications

  1. Korobova F, Ramabhadran V, Higgs H. An actin-dependent step in mitochondrial fission mediated by the ER-associated formin INF2. Science. 2013;339:464-7 pubmed publisher
    ..Because INF2 mutations can lead to Charcot-Marie-Tooth disease, our results provide a potential cellular mechanism for this disease state...
  2. Moreno Ulloa A, Cid A, Rubio Gayosso I, Ceballos G, Villarreal F, Ramirez Sanchez I. Effects of (-)-epicatechin and derivatives on nitric oxide mediated induction of mitochondrial proteins. Bioorg Med Chem Lett. 2013;23:4441-6 pubmed publisher
    ..The effects noted for (-)-epicatechin may serve to explain the healthy effects on cardiometabolic risk ascribed to the consumption of cocoa products. ..
  3. Melser S, Chatelain E, Lavie J, Mahfouf W, Jose C, Obre E, et al. Rheb regulates mitophagy induced by mitochondrial energetic status. Cell Metab. 2013;17:719-30 pubmed publisher
    ..Our data suggest that mitochondrial degradation contributes to a bulk renewal of the organelle in order to prevent mitochondrial aging and to maintain the efficiency of oxidative phosphorylation. ..
  4. Ferree A, Trudeau K, Zik E, Benador I, Twig G, Gottlieb R, et al. MitoTimer probe reveals the impact of autophagy, fusion, and motility on subcellular distribution of young and old mitochondrial protein and on relative mitochondrial protein age. Autophagy. 2013;9:1887-96 pubmed publisher
    ..Collectively our data show that distribution of young and old protein in the mitochondrial network is dependent on turnover, fusion, and transport. ..
  5. Hernandez G, Thornton C, Stotland A, Lui D, Sin J, Ramil J, et al. MitoTimer: a novel tool for monitoring mitochondrial turnover. Autophagy. 2013;9:1852-61 pubmed publisher
    ..Coupled with organelle flow cytometry, it offers new opportunities to investigate mitochondrial subpopulations by biochemical or proteomic methods. ..
  6. Mizunoya W, Sawano S, Iwamoto Y, Sato Y, Tatsumi R, Ikeuchi Y. Effect of 48-h food deprivation on the expressions of myosin heavy-chain isoforms and fiber type-related factors in rats. J Nutr Sci Vitaminol (Tokyo). 2013;59:289-98 pubmed
    ..In conclusion, 48-h food deprivation increased mRNA expression of fast-type MyHC isoform and oxidative metabolism-related factors in EDL, whereas MyHC composition at the protein level did not change in either the soleus or EDL. ..
  7. Jeong S, Xiao C, Finley L, Lahusen T, Souza A, Pierce K, et al. SIRT4 has tumor-suppressive activity and regulates the cellular metabolic response to DNA damage by inhibiting mitochondrial glutamine metabolism. Cancer Cell. 2013;23:450-63 pubmed publisher
    ..Our data uncover SIRT4 as an important component of the DNA damage response pathway that orchestrates a metabolic block in glutamine metabolism, cell cycle arrest, and tumor suppression. ..
  8. Zhang K, Li Z, Jaiswal M, Bayat V, Xiong B, Sandoval H, et al. The C8ORF38 homologue Sicily is a cytosolic chaperone for a mitochondrial complex I subunit. J Cell Biol. 2013;200:807-20 pubmed publisher
    ..Our data indicate that cytosolic chaperones are required for the subcellular transport of ND42. ..
  9. Surovtseva Y, Shadel G. Transcription-independent role for human mitochondrial RNA polymerase in mitochondrial ribosome biogenesis. Nucleic Acids Res. 2013;41:2479-88 pubmed publisher
    ..The significance of these results is discussed with regard to the mechanism and regulation of human mitochondrial gene expression and the potential multi-functionality of RNA polymerases in general. ..

Scientific Experts

More Information


  1. Hashimi H, McDonald L, Stríbrná E, Lukes J. Trypanosome Letm1 protein is essential for mitochondrial potassium homeostasis. J Biol Chem. 2013;288:26914-25 pubmed publisher
    ..Furthermore, although mitochondrial translation is affected upon Letm1 ablation, it is an indirect consequence of K(+) accumulation in the matrix. ..
  2. Qiu J, Wenz L, Zerbes R, Oeljeklaus S, Bohnert M, Stroud D, et al. Coupling of mitochondrial import and export translocases by receptor-mediated supercomplex formation. Cell. 2013;154:596-608 pubmed publisher
    ..Our study reveals receptor-mediated coupling of import and export translocases as a means of precursor channeling. ..
  3. Shinmura K. Post-translational modification of mitochondrial proteins by caloric restriction: possible involvement in caloric restriction-induced cardioprotection. Trends Cardiovasc Med. 2013;23:18-25 pubmed publisher
    ..mitochondria play a central role in cardiac reactive oxygen species production, targeted modification of mitochondrial proteins and subsequent improvement in mitochondrial function have the potential for controlling cardiovascular ..
  4. Tews D, Fischer Posovszky P, Fromme T, Klingenspor M, Fischer J, Ruther U, et al. FTO deficiency induces UCP-1 expression and mitochondrial uncoupling in adipocytes. Endocrinology. 2013;154:3141-51 pubmed publisher
    ..Further understanding of the signaling pathway connecting FTO with UCP-1 expression might lead to new options for obesity and overweight treatment. ..
  5. Allensworth J, Sauer S, Lyerly H, Morse M, Devi G. Smac mimetic Birinapant induces apoptosis and enhances TRAIL potency in inflammatory breast cancer cells in an IAP-dependent and TNF-?-independent mechanism. Breast Cancer Res Treat. 2013;137:359-71 pubmed publisher
    ..By demonstrating that Birinapant primes cancer cells for death in an IAP-dependent manner, these findings support the development of Smac mimetics for IBC treatment. ..
  6. Eckers E, Petrungaro C, Gross D, Riemer J, Hell K, Deponte M. Divergent molecular evolution of the mitochondrial sulfhydryl:cytochrome C oxidoreductase Erv in opisthokonts and parasitic protists. J Biol Chem. 2013;288:2676-88 pubmed publisher
    ..Nevertheless, the knowledge on mitochondrial protein import in yeast and mammals cannot be generally transferred to all other eukaryotes, and the corresponding pathways, components, and mechanisms remain to be analyzed. ..
  7. Sarraf S, Raman M, Guarani Pereira V, Sowa M, Huttlin E, Gygi S, et al. Landscape of the PARKIN-dependent ubiquitylome in response to mitochondrial depolarization. Nature. 2013;496:372-6 pubmed publisher
    ..These studies provide a resource for understanding how the PINK1-PARKIN pathway re-sculpts the proteome to support mitochondrial homeostasis. ..
  8. Kukat C, Larsson N. mtDNA makes a U-turn for the mitochondrial nucleoid. Trends Cell Biol. 2013;23:457-63 pubmed publisher
    ..In this review the structure of the mitochondrial nucleoid and its possible regulatory roles in mtDNA expression will be discussed. ..
  9. Fischer M, Horn S, Belkacemi A, Kojer K, Petrungaro C, Habich M, et al. Protein import and oxidative folding in the mitochondrial intermembrane space of intact mammalian cells. Mol Biol Cell. 2013;24:2160-70 pubmed publisher
  10. Lee M, Kim I, Kim Y. Effects of eicosapentaenoic acid and docosahexaenoic acid on uncoupling protein 3 gene expression in C(2)C(12) muscle cells. Nutrients. 2013;5:1660-71 pubmed publisher
    ..AICAR showed additive effects with EPA or DHA on the UCP3 promoter activation. These results indicate that EPA and DHA directly regulate the gene expression of UCP3, potentially through AMPK-mediated pathway in C(2)C(12) muscle cells...
  11. Ferreira Junior J, Bleicher L, Barros M. Her2p molecular modeling, mutant analysis and intramitochondrial localization. Fungal Genet Biol. 2013;60:133-9 pubmed publisher
    ..For instance, her2/ts11 mutant showed its fermentative growth impaired, and poorly rescued by GTF1 indicating that Her2p unknown function in the mitochondria outer membrane affects cell viability. ..
  12. Fu M, St Pierre P, Shankar J, Wang P, Joshi B, Nabi I. Regulation of mitophagy by the Gp78 E3 ubiquitin ligase. Mol Biol Cell. 2013;24:1153-62 pubmed publisher
    ..This study therefore describes a novel role for the ER-associated Gp78 ubiquitin ligase and the Mfn1 mitochondrial fusion factor in mitophagy. ..
  13. Milenkovic D, Matic S, Kühl I, Ruzzenente B, Freyer C, Jemt E, et al. TWINKLE is an essential mitochondrial helicase required for synthesis of nascent D-loop strands and complete mtDNA replication. Hum Mol Genet. 2013;22:1983-93 pubmed publisher
    ..Our data thus suggest that the relative levels of abortive D-loop synthesis versus complete mtDNA replication are regulated and may provide a mechanism to control progression to complete mtDNA replication. ..
  14. Wredenberg A, Lagouge M, Bratic A, Metodiev M, Spåhr H, Mourier A, et al. MTERF3 regulates mitochondrial ribosome biogenesis in invertebrates and mammals. PLoS Genet. 2013;9:e1003178 pubmed publisher
  15. Wrobel L, Trojanowska A, Sztolsztener M, Chacinska A. Mitochondrial protein import: Mia40 facilitates Tim22 translocation into the inner membrane of mitochondria. Mol Biol Cell. 2013;24:543-54 pubmed publisher
    ..We propose that Mia40 not only is responsible for disulfide bond formation, but also assists the Tim22 protein in its integration into the inner membrane of mitochondria. ..
  16. Steinhart L, Belz K, Fulda S. Smac mimetic and demethylating agents synergistically trigger cell death in acute myeloid leukemia cells and overcome apoptosis resistance by inducing necroptosis. Cell Death Dis. 2013;4:e802 pubmed publisher
    ..The identification of a novel synergism of BV6 and demethylating agents has important implications for the development of new treatment strategies for AML. ..
  17. Richter V, Palmer C, Osellame L, Singh A, Elgass K, Stroud D, et al. Structural and functional analysis of MiD51, a dynamin receptor required for mitochondrial fission. J Cell Biol. 2014;204:477-86 pubmed publisher
    ..MiD51 foci are also dependent on the presence of Drp1, and after scission they are distributed to daughter organelles, supporting the involvement of MiD51 in the fission apparatus...
  18. Srivastava S, Baxa U, Niu G, Chen X, Veech R. A ketogenic diet increases brown adipose tissue mitochondrial proteins and UCP1 levels in mice. IUBMB Life. 2013;65:58-66 pubmed publisher
    ..5-fold higher, suggesting increased sympathetic system activity. These results demonstrate that a KD can also increase BAT mitochondrial size and protein levels. ..
  19. Varabyova A, Topf U, Kwiatkowska P, Wrobel L, Kaus Drobek M, Chacinska A. Mia40 and MINOS act in parallel with Ccs1 in the biogenesis of mitochondrial Sod1. FEBS J. 2013;280:4943-59 pubmed publisher
    ..Thus, we identified novel mitochondrial players that are possibly involved in pathological conditions caused by changes in the biogenesis of Sod1. ..
  20. Sugiura A, Nagashima S, Tokuyama T, Amo T, Matsuki Y, Ishido S, et al. MITOL regulates endoplasmic reticulum-mitochondria contacts via Mitofusin2. Mol Cell. 2013;51:20-34 pubmed publisher
    ..A K192R mutation blocked oligomerization even in the presence of GTP. Taken together, these results suggested that MITOL regulates ER tethering to mitochondria by activating Mfn2 via K192 ubiquitination. ..
  21. Jiang D, Zhao L, Clish C, Clapham D. Letm1, the mitochondrial Ca2+/H+ antiporter, is essential for normal glucose metabolism and alters brain function in Wolf-Hirschhorn syndrome. Proc Natl Acad Sci U S A. 2013;110:E2249-54 pubmed publisher
    ..We conclude that loss of Letm1 contributes to the pathology of Wolf-Hirschhorn syndrome in humans and may contribute to seizure phenotypes by reducing glucose oxidation and other specific metabolic alterations. ..
  22. Weber T, Koob S, Heide H, Wittig I, Head B, VAN DER BLIEK A, et al. APOOL is a cardiolipin-binding constituent of the Mitofilin/MINOS protein complex determining cristae morphology in mammalian mitochondria. PLoS ONE. 2013;8:e63683 pubmed publisher
    ..Our findings further assign an intracellular role to a member of the apolipoprotein family in mammals. ..
  23. Condon S, Mitsuuchi Y, Deng Y, LaPorte M, Rippin S, Haimowitz T, et al. Birinapant, a smac-mimetic with improved tolerability for the treatment of solid tumors and hematological malignancies. J Med Chem. 2014;57:3666-77 pubmed publisher
    ..The P2' position of 1 was critical to this differential activity, and this improved tolerability has allowed 1 to proceed into clinical studies. ..
  24. Itoh K, Tamura Y, Iijima M, Sesaki H. Effects of Fcj1-Mos1 and mitochondrial division on aggregation of mitochondrial DNA nucleoids and organelle morphology. Mol Biol Cell. 2013;24:1842-51 pubmed publisher
    ..that control nucleoid distribution and size, we identify Fcj1 and Mos1, two evolutionarily conserved mitochondrial proteins that maintain the connection between the cristae and boundary membranes...
  25. Fröhlich C, Grabiger S, Schwefel D, Faelber K, Rosenbaum E, Mears J, et al. Structural insights into oligomerization and mitochondrial remodelling of dynamin 1-like protein. EMBO J. 2013;32:1280-92 pubmed publisher
    ..Based on these results and electron microscopy reconstructions, we propose an oligomerization mode for DNM1L which differs from that of dynamin and might be adapted to the remodelling of mitochondria. ..
  26. Tchoghandjian A, Jennewein C, Eckhardt I, Rajalingam K, Fulda S. Identification of non-canonical NF-?B signaling as a critical mediator of Smac mimetic-stimulated migration and invasion of glioblastoma cells. Cell Death Dis. 2013;4:e564 pubmed publisher
  27. McStay G, Su C, Tzagoloff A. Modular assembly of yeast cytochrome oxidase. Mol Biol Cell. 2013;24:440-52 pubmed publisher
    ..Unlike their bacterial counterparts, which are composed only of the individual core subunits, the final sequence in which the mitochondrial modules associate to form the holoenzyme may have been conserved during evolution. ..
  28. Losón O, Song Z, Chen H, Chan D. Fis1, Mff, MiD49, and MiD51 mediate Drp1 recruitment in mitochondrial fission. Mol Biol Cell. 2013;24:659-67 pubmed publisher
    ..Finally, we find that either MiD49 or MiD51 can mediate Drp1 recruitment and mitochondrial fission in the absence of Fis1 and Mff. These results demonstrate that multiple receptors can recruit Drp1 to mediate mitochondrial fission. ..
  29. Csibi A, Fendt S, Li C, Poulogiannis G, Choo A, Chapski D, et al. The mTORC1 pathway stimulates glutamine metabolism and cell proliferation by repressing SIRT4. Cell. 2013;153:840-54 pubmed publisher
    ..Finally, our data indicate that targeting nutrient metabolism in energy-addicted cancers with high mTORC1 signaling may be an effective therapeutic approach. ..
  30. Baker M, Lampe P, Stojanovski D, Korwitz A, Anand R, Tatsuta T, et al. Stress-induced OMA1 activation and autocatalytic turnover regulate OPA1-dependent mitochondrial dynamics. EMBO J. 2014;33:578-93 pubmed publisher
    ..This differentiated stress response maintains the functional integrity of mitochondria and contributes to cell survival. ..
  31. Mick D, Dennerlein S, Wiese H, Reinhold R, Pacheu Grau D, Lorenzi I, et al. MITRAC links mitochondrial protein translocation to respiratory-chain assembly and translational regulation. Cell. 2012;151:1528-41 pubmed publisher
    ..We establish an unexpected molecular link between the TIM23 transport machinery and assembly of respiratory-chain complexes that regulate mitochondrial protein synthesis in response to their assembly state. ..
  32. Jeong S, Lee A, Lee J, Haigis M. SIRT4 protein suppresses tumor formation in genetic models of Myc-induced B cell lymphoma. J Biol Chem. 2014;289:4135-44 pubmed publisher
    ..Together, these results highlight the tumor-suppressive role of SIRT4 in Myc-induced B cell lymphoma and suggest that SIRT4 may be a potential target against Myc-induced and/or glutamine-dependent cancers. ..
  33. Ruan Y, Li H, Zhang K, Jian F, Tang J, Song Z. Loss of Yme1L perturbates mitochondrial dynamics. Cell Death Dis. 2013;4:e896 pubmed publisher
    ..In addition, there is interaction between SLP-2 with Yme1L and shYme1L cells retain stress-induced mitochondrial hyperfusion. Taken together, our results clarify how Yme1L regulates mitochondrial morphology. ..
  34. Bui H, Shaw J. Dynamin assembly strategies and adaptor proteins in mitochondrial fission. Curr Biol. 2013;23:R891-9 pubmed publisher
    ..Here, we review the structure and assembly properties of the yeast and mammalian mitochondrial dynamins and discuss what is known about the activities of their adaptor proteins. ..
  35. Miyake N, Yano S, Sakai C, Hatakeyama H, Matsushima Y, Shiina M, et al. Mitochondrial complex III deficiency caused by a homozygous UQCRC2 mutation presenting with neonatal-onset recurrent metabolic decompensation. Hum Mutat. 2013;34:446-52 pubmed publisher
    ..This is the first described human disease caused by a core protein abnormality in mitochondrial CIII. ..
  36. Palmer C, Elgass K, Parton R, Osellame L, Stojanovski D, Ryan M. Adaptor proteins MiD49 and MiD51 can act independently of Mff and Fis1 in Drp1 recruitment and are specific for mitochondrial fission. J Biol Chem. 2013;288:27584-93 pubmed publisher
    ..We conclude that MiD49 and MiD51 can act independently of Mff and Fis1 in Drp1 recruitment and suggest that they provide specificity to the division of mitochondria. ..
  37. Rubio Cosials A, Sola M. U-turn DNA bending by human mitochondrial transcription factor A. Curr Opin Struct Biol. 2013;23:116-24 pubmed publisher
    ..Finally, the structural localization of disease-related TFAM mutations suggests functional impairment at the molecular level. ..
  38. Bakala H, Ladouce R, Baraibar M, Friguet B. Differential expression and glycative damage affect specific mitochondrial proteins with aging in rat liver. Biochim Biophys Acta. 2013;1832:2057-67 pubmed publisher
  39. Hayward D, Dosztanyi Z, Clark Walker G. The N-terminal intrinsically disordered domain of Mgm101p is localized to the mitochondrial nucleoid. PLoS ONE. 2013;8:e56465 pubmed publisher
    ..By attaching green fluorescent protein to the N-terminal disordered domain of yeast Mgm101p we find that GFP is still directed to the mitochondrial nucleoid where full-length Mgm101p-GFP is targeted. ..
  40. Otera H, Ishihara N, Mihara K. New insights into the function and regulation of mitochondrial fission. Biochim Biophys Acta. 2013;1833:1256-68 pubmed publisher
  41. Koirala S, Guo Q, Kalia R, Bui H, Eckert D, Frost A, et al. Interchangeable adaptors regulate mitochondrial dynamin assembly for membrane scission. Proc Natl Acad Sci U S A. 2013;110:E1342-51 pubmed publisher
    ..This result demonstrates that an adaptor protein alters the architecture of a mitochondrial dynamin GTPase polymer in a manner that could facilitate membrane constriction and severing activity. ..
  42. Houtkooper R, Mouchiroud L, Ryu D, Moullan N, Katsyuba E, Knott G, et al. Mitonuclear protein imbalance as a conserved longevity mechanism. Nature. 2013;497:451-7 pubmed publisher
  43. Aly S, Wen J. Applicability of partial characterization of cytochrome oxidase I in identification of forensically important flies (Diptera) from China and Egypt. Parasitol Res. 2013;112:2667-74 pubmed publisher
    ..Therefore, although molecular methods are beneficial especially if the available evidence are immature stage or degraded fragments of insect, we suggest using this fragment only in addition to other identification methods...
  44. Mao K, Wang K, Liu X, Klionsky D. The scaffold protein Atg11 recruits fission machinery to drive selective mitochondria degradation by autophagy. Dev Cell. 2013;26:9-18 pubmed publisher
    ..These data establish a paradigm for selective organelle degradation...
  45. Guja K, Venkataraman K, Yakubovskaya E, Shi H, Mejia E, Hambardjieva E, et al. Structural basis for S-adenosylmethionine binding and methyltransferase activity by mitochondrial transcription factor B1. Nucleic Acids Res. 2013;41:7947-59 pubmed publisher
    ..Together with mutagenesis studies, these data suggest a model for substrate binding and provide insight into the mechanism of methyl transfer, clarifying the role of this factor in an essential process for mitochondrial function. ..
  46. Cagalinec M, Safiulina D, Liiv M, Liiv J, Choubey V, Wareski P, et al. Principles of the mitochondrial fusion and fission cycle in neurons. J Cell Sci. 2013;126:2187-97 pubmed publisher
    ..This knowledge will increase understanding of the dynamic mitochondrial functions in cells and in particular, the pathogenesis of mitochondrial-related neurodegenerative diseases. ..
  47. Nelson C, Li L, Jacoby R, Millar A. Degradation rate of mitochondrial proteins in Arabidopsis thaliana cells. J Proteome Res. 2013;12:3449-59 pubmed publisher
    ..We provide estimates of degradation rate (K(d)) for 224 mitochondrial proteins, showing a range of over 50-fold in K(d)...
  48. Kulawiak B, Höpker J, Gebert M, Guiard B, Wiedemann N, Gebert N. The mitochondrial protein import machinery has multiple connections to the respiratory chain. Biochim Biophys Acta. 2013;1827:612-26 pubmed publisher
    ..This article is part of a Special Issue entitled: Respiratory complex II: Role in cellular physiology and disease. ..
  49. Terzioglu M, Ruzzenente B, Harmel J, Mourier A, Jemt E, López M, et al. MTERF1 binds mtDNA to prevent transcriptional interference at the light-strand promoter but is dispensable for rRNA gene transcription regulation. Cell Metab. 2013;17:618-26 pubmed publisher
    ..Based on these observations, we suggest that MTERF1 does not regulate heavy-strand transcription, but rather acts to block transcription on the opposite strand of mtDNA to prevent transcription interference at the light-strand promoter. ..
  50. Jans D, Wurm C, Riedel D, Wenzel D, Stagge F, Deckers M, et al. STED super-resolution microscopy reveals an array of MINOS clusters along human mitochondria. Proc Natl Acad Sci U S A. 2013;110:8936-41 pubmed publisher
  51. Vincow E, Merrihew G, Thomas R, Shulman N, Beyer R, MacCoss M, et al. The PINK1-Parkin pathway promotes both mitophagy and selective respiratory chain turnover in vivo. Proc Natl Acad Sci U S A. 2013;110:6400-5 pubmed publisher
    ..Failure to degrade damaged RC proteins could account for the RC deficits seen in many PD patients and may play an important role in PD pathogenesis. ..
  52. Laurent G, German N, Saha A, de Boer V, Davies M, Koves T, et al. SIRT4 coordinates the balance between lipid synthesis and catabolism by repressing malonyl CoA decarboxylase. Mol Cell. 2013;50:686-98 pubmed publisher
    ..In sum, this work elucidates SIRT4 as an important regulator of lipid homeostasis, identifies MCD as a SIRT4 target, and deepens our understanding of the malonyl CoA regulatory axis. ..
  53. Camejo D, Romero Puertas M, Rodríguez Serrano M, Sandalio L, Lázaro J, Jimenez A, et al. Salinity-induced changes in S-nitrosylation of pea mitochondrial proteins. J Proteomics. 2013;79:87-99 pubmed publisher