Genomes and Genes
Experts and Doctors on mitochondrial proteins in Germany
Topic: mitochondrial proteins
Publications183 found, 100 shown here
- Huntgeburth M, Tiemann K, Shahverdyan R, Schluter K, Schreckenberg R, Gross M, et al. Transforming growth factor ?? oppositely regulates the hypertrophic and contractile response to ?-adrenergic stimulation in the heart. PLoS ONE. 2011;6:e26628 pubmed publisher..blockers may act beneficially by normalizing regulatory mechanisms of cellular hypertrophy and energy metabolism. ..
- Zerbes R, HÃ¶ÃŸ P, Pfanner N, van der Laan M, Bohnert M. Distinct Roles of Mic12 and Mic27 in the Mitochondrial Contact Site and Cristae Organizing System. J Mol Biol. 2016;428:1485-92 pubmed publisher..We conclude that in addition to the MICOS core components Mic10 and Mic60, Mic12 and Mic27 play specific roles in the organization of the MICOS complex. ..
- Tarasenko D, Barbot M, Jans D, Kroppen B, Sadowski B, Heim G, et al. The MICOS component Mic60 displays a conserved membrane-bending activity that is necessary for normal cristae morphology. J Cell Biol. 2017;216:889-899 pubmed publisher..Our results show that membrane bending by Mic60 is an ancient mechanism, important for cristae formation, and had already evolved before Î±-proteobacteria developed into mitochondria. ..
- Ehmke N, Graul Neumann L, Smorag L, Koenig R, Segebrecht L, Magoulas P, et al. De Novo Mutations in SLC25A24 Cause a Craniosynostosis Syndrome with Hypertrichosis, Progeroid Appearance, and Mitochondrial Dysfunction. Am J Hum Genet. 2017;101:833-843 pubmed publisher..Our results suggest that the SLC25A24 mutations induce a gain of pathological function and link mitochondrial ATP-Mg/Pi transport to the development of skeletal and connective tissue. ..
- Kubitz R, Helmer A, Haussinger D. Biliary transport systems: short-term regulation. Methods Enzymol. 2005;400:542-57 pubmed..Different techniques have been employed in studies on transporter retrieval and insertion, which are discussed in this chapter. ..
- Sheftel A, Stehling O, Pierik A, Netz D, Kerscher S, Elsässer H, et al. Human ind1, an iron-sulfur cluster assembly factor for respiratory complex I. Mol Cell Biol. 2009;29:6059-73 pubmed publisher..Together, these data identify huInd1 as a new assembly factor for human respiratory complex I with a possible role in the delivery of one or more Fe/S clusters to complex I subunits. ..
- Strodtkötter I, Padmasree K, Dinakar C, Speth B, Niazi P, Wojtera J, et al. Induction of the AOX1D isoform of alternative oxidase in A. thaliana T-DNA insertion lines lacking isoform AOX1A is insufficient to optimize photosynthesis when treated with antimycin A. Mol Plant. 2009;2:284-97 pubmed publisher..We suggest that the aox1a mutants can further be used to substantiate the current models concerning the influence of mitochondrial redox on photosynthetic performance and gene expression. ..
- Hanschmann E, Lönn M, Schütte L, Funke M, Godoy J, Eitner S, et al. Both thioredoxin 2 and glutaredoxin 2 contribute to the reduction of the mitochondrial 2-Cys peroxiredoxin Prx3. J Biol Chem. 2010;285:40699-705 pubmed publisher..These results introduce Grx2 as a novel electron donor for Prx3, providing further insights into pivotal cellular redox signaling mechanisms. ..
- Scheckhuber C, Houthoofd K, Weil A, Werner A, De Vreese A, Vanfleteren J, et al. Alternative oxidase dependent respiration leads to an increased mitochondrial content in two long-lived mutants of the aging model Podospora anserina. PLoS ONE. 2011;6:e16620 pubmed publisher..anserina is linked to a novel mechanism involved in the retrograde response pathway, mitochondrial biogenesis, which might also play an important role for cellular maintenance in other organisms. ..
- Dröse S, Krack S, Sokolova L, Zwicker K, Barth H, Morgner N, et al. Functional dissection of the proton pumping modules of mitochondrial complex I. PLoS Biol. 2011;9:e1001128 pubmed publisher..Our results provide evidence that the membrane arm of complex I harbours two functionally distinct pump modules that are connected in series by the long helical transmission element recently identified by X-ray structural analysis. ..
- Wilfling F, Weber A, Potthoff S, Vögtle F, Meisinger C, Paschen S, et al. BH3-only proteins are tail-anchored in the outer mitochondrial membrane and can initiate the activation of Bax. Cell Death Differ. 2012;19:1328-36 pubmed publisher..The recognition of C-terminal membrane-insertion of BH3-only proteins will permit the development of a more detailed concept of the initiation of mitochondrial apoptosis. ..
- WÃ¼st R, Maurer B, Hauser K, Woitalla D, Sharma M, KrÃ¼ger R. Mutation analyses and association studies to assess the role of the presenilin-associated rhomboid-like gene in Parkinson's disease. Neurobiol Aging. 2016;39:217.e13-5 pubmed publisher..Our findings indicate that PARL mutations are a rare cause of PD and genetic variants are neither strong nor common risk factors in PD. ..
- Gerges S, Rohde K, Fulda S. Cotreatment with Smac mimetics and demethylating agents induces both apoptotic and necroptotic cell death pathways in acute lymphoblastic leukemia cells. Cancer Lett. 2016;375:127-132 pubmed publisher..This opens new perspectives to overcome apoptosis resistance with important implications for the development of new treatment strategies for ALL. ..
- Haberle J, Schmidt E, Pauli S, Kreuder J, Plecko B, Galler A, et al. Mutation analysis in patients with N-acetylglutamate synthase deficiency. Hum Mutat. 2003;21:593-7 pubmed..This is the first report of mutation analysis in a series of families affected with deficiency of NAGS. Molecular analysis of patients and reliable antenatal diagnostics for affected families are now feasible. ..
- Muhlenhoff U, Stadler J, Richhardt N, Seubert A, Eickhorst T, Schweyen R, et al. A specific role of the yeast mitochondrial carriers MRS3/4p in mitochondrial iron acquisition under iron-limiting conditions. J Biol Chem. 2003;278:40612-20 pubmed..Mrs3/4p function in mitochondrial iron transport becomes evident under iron-limiting conditions only, indicating that the two carriers do not represent the sole system for mitochondrial iron acquisition. ..
- Reif S, Randelj O, Domanska G, Dian E, Krimmer T, Motz C, et al. Conserved mechanism of Oxa1 insertion into the mitochondrial inner membrane. J Mol Biol. 2005;354:520-8 pubmed..We conclude that Oxa1 has the ability to accept preproteins within the membrane. ..
- Althaus J, Siegelin M, Dehghani F, Cilenti L, Zervos A, Rami A. The serine protease Omi/HtrA2 is involved in XIAP cleavage and in neuronal cell death following focal cerebral ischemia/reperfusion. Neurochem Int. 2007;50:172-80 pubmed..Blocking the proteolytic activity of Omi/HtrA2 with specific inhibitors, such as the ucf-101, could be a novel way to afford neuroprotection and minimize cellular damage in cerebral ischemia/reperfusion. ..
- Anton F, Fres J, Schauss A, Pinson B, Praefcke G, Langer T, et al. Ugo1 and Mdm30 act sequentially during Fzo1-mediated mitochondrial outer membrane fusion. J Cell Sci. 2011;124:1126-35 pubmed publisher..Our results thus unravel functions of Ugo1 and Mdm30 at distinct steps during OM fusion and suggest that protein clearance confers a non-cycling mechanism to mitofusins, which is distinct from other cellular membrane fusion events. ..
- Wagener N, Ackermann M, Funes S, Neupert W. A pathway of protein translocation in mitochondria mediated by the AAA-ATPase Bcs1. Mol Cell. 2011;44:191-202 pubmed publisher..In bacteria and chloroplasts Rip1 uses the Tat machinery for topogenesis; however, mitochondria have lost this machinery during evolution and a member of the AAA-ATPase family has taken over its function. ..
- Almajan E, Richter R, Paeger L, Martinelli P, Barth E, Decker T, et al. AFG3L2 supports mitochondrial protein synthesis and Purkinje cell survival. J Clin Invest. 2012;122:4048-58 pubmed publisher..We therefore propose that defective mitochondrial protein synthesis, leading to early-onset fragmentation of the mitochondrial network, is a central causative factor in AFG3L2-related neurodegeneration. ..
- Guo H, König R, Deng M, Riess M, Mo J, Zhang L, et al. NLRX1 Sequesters STING to Negatively Regulate the Interferon Response, Thereby Facilitating the Replication of HIV-1 and DNA Viruses. Cell Host Microbe. 2016;19:515-528 pubmed publisher..Accordingly, Nlrx1(-/-) mice infected with DNA viruses exhibit enhanced innate immunity and reduced viral load. Thus, NLRX1 is a negative regulator of the host innate immune response to HIV-1 and DNA viruses. ..
- Herrmann J, Neupert W, Stuart R. Insertion into the mitochondrial inner membrane of a polytopic protein, the nuclear-encoded Oxa1p. EMBO J. 1997;16:2217-26 pubmed..We postulate that at least two different sorting pathways exist in mitochondria for polytopic inner membrane proteins, the evolutionarily novel pathway for members of the ADP/ATP carrier family and a conserved Oxa1p-type pathway. ..
- Lode A, Kuschel M, Paret C, Rödel G. Mitochondrial copper metabolism in yeast: interaction between Sco1p and Cox2p. FEBS Lett. 2000;485:19-24 pubmed..These amino acids, which are conserved among the members of the Sco1p family, have been suggested to act in the reduction of the cysteines in the copper binding center of Cox2p and are discussed as ligands for copper. ..
- Perwitz N, Fasshauer M, Klein J. Cannabinoid receptor signaling directly inhibits thermogenesis and alters expression of adiponectin and visfatin. Horm Metab Res. 2006;38:356-8 pubmed
- Klodmann J, Senkler M, Rode C, Braun H. Defining the protein complex proteome of plant mitochondria. Plant Physiol. 2011;157:587-98 pubmed publisher..complexes including pentatricopeptide repeat proteins involved in nucleic acid metabolism). Discovery of further protein complexes within our data set is open to everybody via the public GelMap portal at www.gelmap.de/arabidopsis_mito. ..
- Gess B, Schirmacher A, Boentert M, Young P. Charcot-Marie-Tooth disease: frequency of genetic subtypes in a German neuromuscular center population. Neuromuscul Disord. 2013;23:647-51 pubmed publisher..This study provides further data on frequencies of CMT genes and subtypes and points to the importance of a thorough clinical and electrophysiological work-up for the direction of genetic testing. ..
- 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. ..
- Lang A, Grether Beck S, Singh M, Kuck F, Jakob S, Kefalas A, et al. MicroRNA-15b regulates mitochondrial ROS production and the senescence-associated secretory phenotype through sirtuin 4/SIRT4. Aging (Albany NY). 2016;8:484-505 pubmed..Thus, miR-15b is a negative regulator of stress-induced SIRT4 expression thereby counteracting senescence associated mitochondrial dysfunction and regulating the SASP and possibly organ aging, such as photoaging of human skin. ..
- Zabezhinsky D, Slobodin B, Rapaport D, Gerst J. An Essential Role for COPI in mRNA Localization to Mitochondria and Mitochondrial Function. Cell Rep. 2016;15:540-549 pubmed publisher..Overall, COPI-mediated mMP targeting is critical for mitochondrial protein import and function, and transcript delivery to the mitochondria or endoplasmic reticulum is regulated by cis-acting RNA sequences and trans-acting proteins. ..
- Oxelmark E, Marchini A, Malanchi I, Magherini F, Jaquet L, Hajibagheri M, et al. Mmf1p, a novel yeast mitochondrial protein conserved throughout evolution and involved in maintenance of the mitochondrial genome. Mol Cell Biol. 2000;20:7784-97 pubmed..5, like Mmf1p, is a soluble protein of the matrix. Our study identifies a biological function for Mmf1p and furthermore indicates that this function is conserved between members of the p14.5 family. ..
- Wiedemann N, Truscott K, Pfannschmidt S, Guiard B, Meisinger C, Pfanner N. Biogenesis of the protein import channel Tom40 of the mitochondrial outer membrane: intermembrane space components are involved in an early stage of the assembly pathway. J Biol Chem. 2004;279:18188-94 pubmed..We propose that the biogenesis pathway of beta-barrel proteins of the outer mitochondrial membrane not only requires TOM and SAM components, but also involves components of the intermembrane space. ..
- Koch M, Breithaupt C, Kiefersauer R, Freigang J, Huber R, Messerschmidt A. Crystal structure of protoporphyrinogen IX oxidase: a key enzyme in haem and chlorophyll biosynthesis. EMBO J. 2004;23:1720-8 pubmed..This modelled transmembrane complex provides a structural explanation for the uncoupling of haem biosynthesis observed in variegate porphyria patients and in plants after inhibiting PPO. ..
- Yan Y, Mahotka C, Heikaus S, Shibata T, Wethkamp N, Liebmann J, et al. Disturbed balance of expression between XIAP and Smac/DIABLO during tumour progression in renal cell carcinomas. Br J Cancer. 2004;91:1349-57 pubmed
- Schrader M, Fahimi H. Growth and division of peroxisomes. Int Rev Cytol. 2006;255:237-90 pubmed..Special emphasis is on the function of dynamin-related proteins (DRPs), on Fis1, a putative adaptor for DRPs, on the role of the Pex11 family of peroxisomal membrane proteins, and the cytoskeleton. ..
- Fromme T, Reichwald K, Platzer M, Li X, Klingenspor M. Chicken ovalbumin upstream promoter transcription factor II regulates uncoupling protein 3 gene transcription in Phodopus sungorus. BMC Mol Biol. 2007;8:1 pubmed..We therefore investigated whether Coup-TFII is a further player in the transcriptional control of the Ucp3 gene in rodents...
- Wittig I, Karas M, Schagger H. High resolution clear native electrophoresis for in-gel functional assays and fluorescence studies of membrane protein complexes. Mol Cell Proteomics. 2007;6:1215-25 pubmed..The advantages of high resolution clear native electrophoresis make this technique superior for functional proteomics analyses. ..
- Reinders J, Wagner K, Zahedi R, Stojanovski D, Eyrich B, van der Laan M, et al. Profiling phosphoproteins of yeast mitochondria reveals a role of phosphorylation in assembly of the ATP synthase. Mol Cell Proteomics. 2007;6:1896-906 pubmed..The authentic phosphoproteome of yeast mitochondria will represent a rich source to uncover novel roles of reversible protein phosphorylation. ..
- Stojanovski D, Guiard B, Kozjak Pavlovic V, Pfanner N, Meisinger C. Alternative function for the mitochondrial SAM complex in biogenesis of alpha-helical TOM proteins. J Cell Biol. 2007;179:881-93 pubmed..Thus, the substrate specificity of SAM is not restricted to beta-barrel proteins but also includes the majority of alpha-helical Tom proteins. ..
- Mourier A, Larsson N. Tracing the trail of protons through complex I of the mitochondrial respiratory chain. PLoS Biol. 2011;9:e1001129 pubmed publisher..In this Primer, we will briefly introduce the recent progress made in this area and highlight the road ahead that likely will unravel the detailed molecular mechanisms of complex I function. ..
- Marchini A, Accardi R, Malanchi I, Schyr E, Oxelmark E, De Pinto V, et al. Schizosaccharomyces pombe Pmf1p is structurally and functionally related to Mmf1p of Saccharomyces cerevisiae. Yeast. 2002;19:703-11 pubmed..Thus, these data together with our previous study show that the mitochondrial function(s) of the p14.5 family members are highly conserved in eukaryotic cells. ..
- Naumann B, Busch A, Allmer J, Ostendorf E, Zeller M, Kirchhoff H, et al. Comparative quantitative proteomics to investigate the remodeling of bioenergetic pathways under iron deficiency in Chlamydomonas reinhardtii. Proteomics. 2007;7:3964-79 pubmed
- Nieto Jacobo F, Pasch D, Basse C. The mitochondrial Dnm1-like fission component is required for lgA2-induced mitophagy but dispensable for starvation-induced mitophagy in Ustilago maydis. Eukaryot Cell. 2012;11:1154-66 pubmed publisher..In conclusion, our results specify the role of the mitochondrial fission factor Dnm1 in mitophagy and uncover differences between mitophagy pathways in the same cellular system. ..
- 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. ..
- Klecker T, Wemmer M, Haag M, Weig A, BÃ¶ckler S, Langer T, et al. Interaction of MDM33 with mitochondrial inner membrane homeostasis pathways in yeast. Sci Rep. 2015;5:18344 pubmed publisher..Our data suggest that MDM33 functionally interacts with components important for inner membrane homeostasis and thereby supports mitochondrial division. ..
- Krause Buchholz U, Tzschoppe K, Paret C, Ostermann K, Rödel G. Identification of functionally important regions of the Saccharomyces cerevisiae mitochondrial translational activator Cbs1p. Yeast. 2000;16:353-63 pubmed..We further show that both short hydrophobic regions are essential for Cbs1p function, although membrane association is observed even in the absence of these regions. ..
- Wagner K, Mick D, Rehling P. Protein transport machineries for precursor translocation across the inner mitochondrial membrane. Biochim Biophys Acta. 2009;1793:52-9 pubmed publisher..Here we describe the organization of the TIM complexes and discuss the current models as to how they mediate the posttranslational import of proteins across and into the inner mitochondrial membrane. ..
- Sheftel A, Wilbrecht C, Stehling O, Niggemeyer B, Elsässer H, Muhlenhoff U, et al. The human mitochondrial ISCA1, ISCA2, and IBA57 proteins are required for [4Fe-4S] protein maturation. Mol Biol Cell. 2012;23:1157-66 pubmed publisher..Taken together, our data suggest that ISCA1, ISCA2, and IBA57 are specifically involved in the maturation of mitochondrial [4Fe-4S] proteins functioning late in the ISC assembly pathway. ..
- MÃ¼ller C, Bildl W, Haupt A, Ellenrieder L, Becker T, Hunte C, et al. Cryo-slicing Blue Native-Mass Spectrometry (csBN-MS), a Novel Technology for High Resolution Complexome Profiling. Mol Cell Proteomics. 2016;15:669-81 pubmed publisher..Together, cryo-slicing BN-MS enables quantitative profiling of complexomes with resolution close to the limits of native gel electrophoresis. ..
- Klepac K, KiliÄ‡ A, Gnad T, Brown L, Herrmann B, Wilderman A, et al. The Gq signalling pathway inhibits brown and beige adipose tissue. Nat Commun. 2016;7:10895 pubmed publisher..Thus, our data indicate that Gq signalling regulates brown/beige adipocytes and inhibition of Gq signalling may be a novel therapeutic approach to combat obesity. ..
- Schwenk R, Luiken J, Eckel J. FIP2 and Rip11 specify Rab11a-mediated cellular distribution of GLUT4 and FAT/CD36 in H9c2-hIR cells. Biochem Biophys Res Commun. 2007;363:119-25 pubmed..These data therefore show that (i) Rab11a regulates cell surface abundance of both GLUT4 and FAT/CD36 and that (ii) both Rab11a-dependent processes are differently regulated by Rab11a effector proteins. ..
- Kieper N, HolmstrÃ¶m K, Ciceri D, Fiesel F, Wolburg H, Ziviani E, et al. Modulation of mitochondrial function and morphology by interaction of Omi/HtrA2 with the mitochondrial fusion factor OPA1. Exp Cell Res. 2010;316:1213-24 pubmed publisher..Our results underscore a critical role of impaired mitochondrial dynamics in neurodegenerative disorders. ..
- Hunte C, Zickermann V, Brandt U. Functional modules and structural basis of conformational coupling in mitochondrial complex I. Science. 2010;329:448-51 pubmed publisher..We suggest that a approximately 60-angstrom-long helical transmission element is critical for transducing conformational energy to proton-pumping elements in the distal module of the membrane arm. ..
- Eckardt K, Schober A, Platzbecker B, Mracek T, Bing C, Trayhurn P, et al. The adipokine zinc-?2-glycoprotein activates AMP kinase in human primary skeletal muscle cells. Arch Physiol Biochem. 2011;117:88-93 pubmed publisher..This mechanism may be involved in mediating the effects of ZAG in relation to increased energy utilization. ..
- Papic D, Krumpe K, Dukanovic J, Dimmer K, Rapaport D. Multispan mitochondrial outer membrane protein Ugo1 follows a unique Mim1-dependent import pathway. J Cell Biol. 2011;194:397-405 pubmed publisher..Collectively, these results suggest that Ugo1 is inserted into the MOM by a novel pathway in which Tom70 and Mim1 contribute to the efficiency and selectivity of the process. ..
- Jaksch M, Horvath R, Horn N, Auer D, MacMillan C, Peters J, et al. Homozygosity (E140K) in SCO2 causes delayed infantile onset of cardiomyopathy and neuropathy. Neurology. 2001;57:1440-6 pubmed..There is increased copper uptake in patients' fibroblasts indicating that the G1541A mutation effects cellular copper metabolism. ..
- 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. ..
- Vogler S, Goedde R, Miterski B, Gold R, Kroner A, Koczan D, et al. Association of a common polymorphism in the promoter of UCP2 with susceptibility to multiple sclerosis. J Mol Med (Berl). 2005;83:806-11 pubmed..Thus, UCP2 promoter polymorphism may contribute to MS susceptibility by regulating the level of UCP2 protein in the central nervous and/or the immune systems. ..
- Netz D, Stümpfig M, Dore C, Muhlenhoff U, Pierik A, Lill R. Tah18 transfers electrons to Dre2 in cytosolic iron-sulfur protein biogenesis. Nat Chem Biol. 2010;6:758-65 pubmed publisher..The pathway is conserved in eukaryotes, as human Ndor1-Ciapin1 proteins can functionally replace yeast Tah18-Dre2. ..
- Castex J, Willmann D, Kanouni T, Arrigoni L, Li Y, Friedrich M, et al. Inactivation of Lsd1 triggers senescence in trophoblast stem cells by induction of Sirt4. Cell Death Dis. 2017;8:e2631 pubmed publisher..In conclusion, by repression of Sirt4, Lsd1 directs the epigenetic control of TSC immortality via maintenance of metabolic flexibility. ..
- Rehling P, Wiedemann N, Pfanner N, Truscott K. The mitochondrial import machinery for preproteins. Crit Rev Biochem Mol Biol. 2001;36:291-336 pubmed..Moreover, we are now approaching a new era in which elaborated techniques have already allowed and will enable us to gather information about the TOM and TIM complexes on an ultrastructural level. ..
- Habib S, Waizenegger T, Lech M, Neupert W, Rapaport D. Assembly of the TOB complex of mitochondria. J Biol Chem. 2005;280:6434-40 pubmed..Mas37 assembled directly with the TOB core complex. Hence, the biogenesis of Mas37 represents a novel import pathway of mitochondrial proteins. ..
- Thinh V, Hallam C, Roos C, Hammerschmidt K. Concordance between vocal and genetic diversity in crested gibbons. BMC Evol Biol. 2011;11:36 pubmed publisher..As vocal production in other nonhuman primate species appears to be evolutionarily based, it is likely that loud calls produced by other species can serve as characters to elucidate phylogenetic relationships. ..
- Rapaport D, Brunner M, Neupert W, Westermann B. Fzo1p is a mitochondrial outer membrane protein essential for the biogenesis of functional mitochondria in Saccharomyces cerevisiae. J Biol Chem. 1998;273:20150-5 pubmed..It is imported into the outer membrane in a receptor-dependent manner. Fzo1p is part of a larger protein complex of 800 kDa, and presumably is the first identified component of the yeast mitochondrial fusion machinery. ..
- Gerber J, Muhlenhoff U, Lill R. An interaction between frataxin and Isu1/Nfs1 that is crucial for Fe/S cluster synthesis on Isu1. EMBO Rep. 2003;4:906-11 pubmed..The iron-dependent binding of Yfh1 to Isu1/Nfs1 suggests a role of frataxin/Yfh1 in iron loading of the Isu scaffold proteins. ..
- Sunderhaus S, Klodmann J, Lenz C, Braun H. Supramolecular structure of the OXPHOS system in highly thermogenic tissue of Arum maculatum. Plant Physiol Biochem. 2010;48:265-72 pubmed publisher..maculatum at the same time has high AOX and I + III(2) supercomplex abundance and activity, negative regulation of the alternative oxidase by supercomplex formation seems not to occur. Functional implications are discussed. ..
- Potting C, Wilmes C, Engmann T, Osman C, Langer T. Regulation of mitochondrial phospholipids by Ups1/PRELI-like proteins depends on proteolysis and Mdm35. EMBO J. 2010;29:2888-98 pubmed publisher..Homologues to all components of this pathway are present in higher eukaryotes, suggesting that the regulation of mitochondrial CL and PE levels is conserved in evolution. ..
- Liu X, Trakooljul N, MurÃ¡ni E, Krischek C, Schellander K, Wicke M, et al. Molecular changes in mitochondrial respiratory activity and metabolic enzyme activity in muscle of four pig breeds with distinct metabolic types. J Bioenerg Biomembr. 2016;48:55-65 pubmed publisher..Overall, the results further the breed-specific understanding of the molecular basis of metabolic enzyme activities, which directly impact meat quality. ..
- Fritz S, Weinbach N, Westermann B. Mdm30 is an F-box protein required for maintenance of fusion-competent mitochondria in yeast. Mol Biol Cell. 2003;14:2303-13 pubmed..Our results suggest that Mdm30 controls mitochondrial shape by regulating the steady-state level of Fzo1 and point to a connection of the ubiquitin/26S proteasome system and mitochondria. ..
- Sanjuán Szklarz L, Guiard B, Rissler M, Wiedemann N, Kozjak V, van der Laan M, et al. Inactivation of the mitochondrial heat shock protein zim17 leads to aggregation of matrix hsp70s followed by pleiotropic effects on morphology and protein biogenesis. J Mol Biol. 2005;351:206-18 pubmed
- Escobar Henriques M, Westermann B, Langer T. Regulation of mitochondrial fusion by the F-box protein Mdm30 involves proteasome-independent turnover of Fzo1. J Cell Biol. 2006;173:645-50 pubmed
- Neschen S, Katterle Y, Richter J, Augustin R, Scherneck S, Mirhashemi F, et al. Uncoupling protein 1 expression in murine skeletal muscle increases AMPK activation, glucose turnover, and insulin sensitivity in vivo. Physiol Genomics. 2008;33:333-40 pubmed publisher..UCP1-mediated uncoupling of oxidative phosphorylation in skeletal muscle was paralleled by AMPK activation and thereby stimulated insulin-mediated glucose uptake in skeletal muscle. ..
- Sheftel A, Lill R. The power plant of the cell is also a smithy: the emerging role of mitochondria in cellular iron homeostasis. Ann Med. 2009;41:82-99 pubmed publisher..The goal of this article is to provide an overview of how mitochondria acquire and use iron and examine the ramifications of disturbances in these processes on overall cellular iron homeostasis. ..
- Mahlert M, Vogler C, Stelter K, Hause G, Basse C. The a2 mating-type-locus gene lga2 of Ustilago maydis interferes with mitochondrial dynamics and fusion, partially in dependence on a Dnm1-like fission component. J Cell Sci. 2009;122:2402-12 pubmed publisher..We conclude that Lga2 acts upstream of Dnm1, but controls mitochondrial integrity independently of Dnm1-mediated fission. In addition, we demonstrate a role of dnm1 in fungal virulence...
- Bundschuh F, Hannappel A, Anderka O, Ludwig B. Surf1, associated with Leigh syndrome in humans, is a heme-binding protein in bacterial oxidase biogenesis. J Biol Chem. 2009;284:25735-41 pubmed publisher..Contrary to most earlier concepts, these data support a direct role of Surf1 in heme a cofactor insertion into COX subunit I by providing a protein-bound heme a pool. ..
- Chromik J, Safferthal C, Serve H, Fulda S. Smac mimetic primes apoptosis-resistant acute myeloid leukaemia cells for cytarabine-induced cell death by triggering necroptosis. Cancer Lett. 2014;344:101-109 pubmed publisher..These findings have important implications for Smac mimetic-based strategies to bypass apoptosis resistance of AML. ..
- 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. ..
- Huang S, Klingenberg M. Chloride channel properties of the uncoupling protein from brown adipose tissue mitochondria: a patch-clamp study. Biochemistry. 1996;35:16806-14 pubmed..The Cl- conductance measured under these conditions is 6 orders of magnitude higher than the Cl- transport activity reported earlier, suggesting that the UCP has the potential of behaving as an anion channel. ..
- Kinner A, Kölling R. The yeast deubiquitinating enzyme Ubp16 is anchored to the outer mitochondrial membrane. FEBS Lett. 2003;549:135-40 pubmed
- Gerber J, Neumann K, Prohl C, Muhlenhoff U, Lill R. The yeast scaffold proteins Isu1p and Isu2p are required inside mitochondria for maturation of cytosolic Fe/S proteins. Mol Cell Biol. 2004;24:4848-57 pubmed..Together, our data suggest that the Isu proteins need to be localized in mitochondria to fulfill their functional requirement in Fe/S protein maturation in the cytosol. ..
- Jastroch M, Withers K, Taudien S, Frappell P, Helwig M, Fromme T, et al. Marsupial uncoupling protein 1 sheds light on the evolution of mammalian nonshivering thermogenesis. Physiol Genomics. 2008;32:161-9 pubmed..Our data suggest that an archetypal brown adipose tissue was present at least 150 million yr ago allowing early mammals to produce endogenous heat in the cold, without dependence on shivering and locomotor activity. ..
- Koppen M, Bonn F, Ehses S, Langer T. Autocatalytic processing of m-AAA protease subunits in mitochondria. Mol Biol Cell. 2009;20:4216-24 pubmed publisher..These findings might be of relevance for the pathogenesis of neurodegenerative disorders associated with mutations in different m-AAA protease subunits. ..
- Osman C, Haag M, Wieland F, Brugger B, Langer T. A mitochondrial phosphatase required for cardiolipin biosynthesis: the PGP phosphatase Gep4. EMBO J. 2010;29:1976-87 pubmed publisher..Our experiments provide the framework for the further dissection of mechanisms that are required for accumulation and maintenance of CL levels in mitochondria. ..
- Kim M, Fekadu J, Maronde E, Rami A. Alleviation of autophagy by knockdown of Beclin-1 enhances susceptibility of hippocampal neurons to proapoptotic signals induced by amino acid starvation. Histochem Cell Biol. 2013;139:99-108 pubmed publisher..Our data further show that attenuation of autophagy by a knockdown of beclin-1 enhanced neurons susceptibility to proapoptotic signals induced by AAS and underlines that autophagy is per se a protective than a deleterious mechanism. ..
- Di Bartolomeo F, Doan K, Athenstaedt K, Becker T, Daum G. Involvement of a putative substrate binding site in the biogenesis and assembly of phosphatidylserine decarboxylase 1 from Saccharomyces cerevisiae. Biochim Biophys Acta Mol Cell Biol Lipids. 2017;1862:716-725 pubmed publisher..Our data show that mutations in this motif affect processing and stability of Psd1p, and consequently the enzyme's activity. Thus, we conclude that this consensus motif is essential for structural integrity and processing of Psd1p. ..
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