Peter Rehling



  1. Dudek J, Hartmann M, Rehling P. The role of mitochondrial cardiolipin in heart function and its implication in cardiac disease. Biochim Biophys Acta Mol Basis Dis. 2019;1865:810-821 pubmed publisher
  2. Richter F, Dennerlein S, Nikolov M, Jans D, Naumenko N, Aich A, et al. ROMO1 is a constituent of the human presequence translocase required for YME1L protease import. J Cell Biol. 2019;218:598-614 pubmed publisher
    ..Our analyses establish an unexpected link between mitochondrial protein import and inner membrane protein quality control. ..
  3. Chowdhury A, Aich A, Jain G, Wozny K, Lüchtenborg C, Hartmann M, et al. Defective Mitochondrial Cardiolipin Remodeling Dampens HIF-1α Expression in Hypoxia. Cell Rep. 2018;25:561-570.e6 pubmed publisher
    ..We conclude that defective mitochondrial cardiolipin remodeling dampens HIF-1α signaling due to a lack of NF-κB activation through reduced mitochondrial ROS production, decreasing HIF-1α transcription. ..
  4. Schendzielorz A, Bragoszewski P, Naumenko N, Gomkale R, Schulz C, Guiard B, et al. Motor recruitment to the TIM23 channel's lateral gate restricts polypeptide release into the inner membrane. Nat Commun. 2018;9:4028 pubmed publisher
    ..This finding explains why a motor-free form of the translocase facilitates the lateral movement of precursors with a stop-transfer signal. ..
  5. Dennerlein S, Rehling P. Human mitochondrial COX1 assembly into cytochrome c oxidase at a glance. J Cell Sci. 2015;128:833-7 pubmed publisher
    ..Furthermore, these findings will be discussed in the context of human COX-related disorders. ..
  6. Lorenzi I, Oeljeklaus S, Ronsör C, Bareth B, Warscheid B, Rehling P, et al. Ribosome-Associated Mba1 Escorts Cox2 from Insertion Machinery to Maturing Assembly Intermediates. Mol Cell Biol. 2016;36:2782-2793 pubmed publisher
    ..We suggest that Mba1 stabilizes the Cox20-ribosome complex and supports the handover of Cox2 to the Cox18 tail export machinery. ..
  7. Dudek J, Cheng I, Chowdhury A, Wozny K, Balleininger M, Reinhold R, et al. Cardiac-specific succinate dehydrogenase deficiency in Barth syndrome. EMBO Mol Med. 2016;8:139-54 pubmed publisher
    ..As a similar defect of SDH is apparent in patient cell-derived cardiomyocytes, we conclude that these defects represent a molecular basis for the cardiac pathology in Barth syndrome. ..
  8. Bareth B, Nikolov M, Lorenzi I, Hildenbeutel M, Mick D, Helbig C, et al. Oms1 associates with cytochrome c oxidase assembly intermediates to stabilize newly synthesized Cox1. Mol Biol Cell. 2016;27:1570-80 pubmed publisher
    ..Our analyses show that Oms1 participates in cytochrome c oxidase assembly by stabilizing newly synthesized Cox1. ..
  9. Levchenko M, Wuttke J, Römpler K, Schmidt B, Neifer K, Juris L, et al. Cox26 is a novel stoichiometric subunit of the yeast cytochrome c oxidase. Biochim Biophys Acta. 2016;1863:1624-32 pubmed publisher
    ..Our analyses reveal Cox26 as a novel stoichiometric structural subunit of the cytochrome c oxidase. A loss of Cox26 affects cytochrome c oxidase activity and respirasome organization. ..

More Information


  1. Melin J, Schulz C, Wrobel L, Bernhard O, Chacinska A, Jahn O, et al. Presequence recognition by the tom40 channel contributes to precursor translocation into the mitochondrial matrix. Mol Cell Biol. 2014;34:3473-85 pubmed publisher
    ..The identified presequence-interacting region of Tom40 is capable of functioning independently of the established trans-acting TOM presequence-binding domain during matrix import. ..
  2. Richter Dennerlein R, Oeljeklaus S, Lorenzi I, Ronsör C, Bareth B, Schendzielorz A, et al. Mitochondrial Protein Synthesis Adapts to Influx of Nuclear-Encoded Protein. Cell. 2016;167:471-483.e10 pubmed publisher
    ..These findings establish a mammalian translational plasticity pathway in mitochondria that enables adaptation of mitochondrial protein synthesis to the influx of nuclear-encoded subunits. ..
  3. Schendzielorz A, Schulz C, Lytovchenko O, Clancy A, Guiard B, Ieva R, et al. Two distinct membrane potential-dependent steps drive mitochondrial matrix protein translocation. J Cell Biol. 2017;216:83-92 pubmed publisher
    ..The ??- and Pam17-dependent import step identified in this study is positioned between the two known energy-dependent steps: ??-driven presequence translocation and adenosine triphosphate-driven import motor activity. ..
  4. Dennerlein S, Wang C, Rehling P. Plasticity of Mitochondrial Translation. Trends Cell Biol. 2017;27:712-721 pubmed publisher
    ..We discuss how mitochondrial translation is affected by the cellular environment and propose models of translational plasticity that modulate mitochondrial translation in response to the availability of imported proteins. ..
  5. Naumenko N, Morgenstern M, Rucktäschel R, Warscheid B, Rehling P. INA complex liaises the F1Fo-ATP synthase membrane motor modules. Nat Commun. 2017;8:1237 pubmed publisher
    ..We conclude that INAC maintains assembly intermediates of the F1 F0-ATP synthase in a primed state for the terminal assembly step-motor module formation. ..
  6. Lorenzi I, Oeljeklaus S, Aich A, Ronsör C, Callegari S, Dudek J, et al. The mitochondrial TMEM177 associates with COX20 during COX2 biogenesis. Biochim Biophys Acta Mol Cell Res. 2018;1865:323-333 pubmed publisher
    ..Our data shows that by unbalancing the amount of TMEM177, newly synthesized COX2 accumulates in a COX20-associated state. We conclude that TMEM177 promotes assembly of COX2 at the level of CuA-site formation. ..
  7. Dennerlein S, Oeljeklaus S, Jans D, Hellwig C, Bareth B, Jakobs S, et al. MITRAC7 Acts as a COX1-Specific Chaperone and Reveals a Checkpoint during Cytochrome c Oxidase Assembly. Cell Rep. 2015;12:1644-55 pubmed publisher
    ..In contrast, MITRAC7 deficiency leads to turnover of newly synthesized COX1. Accordingly, MITRAC7 affects the biogenesis pathway by stabilizing newly synthesized COX1 in assembly intermediates, concomitantly preventing turnover. ..
  8. Pacheu Grau D, Bareth B, Dudek J, Juris L, Vögtle F, Wissel M, et al. Cooperation between COA6 and SCO2 in COX2 maturation during cytochrome c oxidase assembly links two mitochondrial cardiomyopathies. Cell Metab. 2015;21:823-33 pubmed publisher
    ..Our analyses define COA6 as a constituent of the mitochondrial copper relay system, linking defects in COX2 metallation to cardiac cytochrome c oxidase deficiency. ..
  9. Melin J, Kilisch M, Neumann P, Lytovchenko O, Gomkale R, Schendzielorz A, et al. A presequence-binding groove in Tom70 supports import of Mdl1 into mitochondria. Biochim Biophys Acta. 2015;1853:1850-9 pubmed publisher
    ..Hence Tom70 contributes to the presequence import pathway by recognition of the targeting signal of the Mdl1 precursor. ..
  10. Schulz C, Schendzielorz A, Rehling P. Unlocking the presequence import pathway. Trends Cell Biol. 2015;25:265-75 pubmed publisher
    ..Here, we review recent advances in understanding of the mechanisms underlying protein transport along the presequence pathway. ..