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Lethal Interaction of Nuclear and Mitochondrial Genotypes in Drosophila melanogaster.

Salminen TS, Cannino G, Oliveira MT, Lillsunde P, Jacobs HT, Kaguni LS.

G3 (Bethesda). 2019 May 10. pii: g3.400315.2019. doi: 10.1534/g3.119.400315. [Epub ahead of print]


RNase H1 promotes replication fork progression through oppositely transcribed regions of Drosophila mitochondrial DNA.

González de Cózar JM, Gerards M, Teeri E, George J, Dufour E, Jacobs HT, Jõers P.

J Biol Chem. 2019 Mar 22;294(12):4331-4344. doi: 10.1074/jbc.RA118.007015. Epub 2019 Jan 11.


Alternative oxidase-mediated respiration prevents lethal mitochondrial cardiomyopathy.

Rajendran J, Purhonen J, Tegelberg S, Smolander OP, Mörgelin M, Rozman J, Gailus-Durner V, Fuchs H, Hrabe de Angelis M, Auvinen P, Mervaala E, Jacobs HT, Szibor M, Fellman V, Kallijärvi J.

EMBO Mol Med. 2019 Jan;11(1). pii: e9456. doi: 10.15252/emmm.201809456.


Alternative respiratory chain enzymes: Therapeutic potential and possible pitfalls.

Saari S, Garcia GS, Bremer K, Chioda MM, Andjelković A, Debes PV, Nikinmaa M, Szibor M, Dufour E, Rustin P, Oliveira MT, Jacobs HT.

Biochim Biophys Acta Mol Basis Dis. 2019 Apr 1;1865(4):854-866. doi: 10.1016/j.bbadis.2018.10.012. Epub 2018 Oct 17.


Alternative Oxidase Attenuates Cigarette Smoke-induced Lung Dysfunction and Tissue Damage.

Giordano L, Farnham A, Dhandapani PK, Salminen L, Bhaskaran J, Voswinckel R, Rauschkolb P, Scheibe S, Sommer N, Beisswenger C, Weissmann N, Braun T, Jacobs HT, Bals R, Herr C, Szibor M.

Am J Respir Cell Mol Biol. 2019 May;60(5):515-522. doi: 10.1165/rcmb.2018-0261OC.


Expression of the Alternative Oxidase Influences Jun N-Terminal Kinase Signaling and Cell Migration.

Andjelković A, Mordas A, Bruinsma L, Ketola A, Cannino G, Giordano L, Dhandapani PK, Szibor M, Dufour E, Jacobs HT.

Mol Cell Biol. 2018 Nov 28;38(24). pii: e00110-18. doi: 10.1128/MCB.00110-18. Print 2018 Dec 15.


Perturbed Redox Signaling Exacerbates a Mitochondrial Myopathy.

Dogan SA, Cerutti R, Benincá C, Brea-Calvo G, Jacobs HT, Zeviani M, Szibor M, Viscomi C.

Cell Metab. 2018 Nov 6;28(5):764-775.e5. doi: 10.1016/j.cmet.2018.07.012. Epub 2018 Aug 16.


Developmental arrest in Drosophila melanogaster caused by mitochondrial DNA replication defects cannot be rescued by the alternative oxidase.

Rodrigues APC, Camargo AF, Andjelković A, Jacobs HT, Oliveira MT.

Sci Rep. 2018 Jul 18;8(1):10882. doi: 10.1038/s41598-018-29150-x.


Polyadenylation and degradation of structurally abnormal mitochondrial tRNAs in human cells.

Toompuu M, Tuomela T, Laine P, Paulin L, Dufour E, Jacobs HT.

Nucleic Acids Res. 2018 Jun 1;46(10):5209-5226. doi: 10.1093/nar/gky159.


Structural rearrangements in the mitochondrial genome of Drosophila melanogaster induced by elevated levels of the replicative DNA helicase.

Ciesielski GL, Nadalutti CA, Oliveira MT, Jacobs HT, Griffith JD, Kaguni LS.

Nucleic Acids Res. 2018 Apr 6;46(6):3034-3046. doi: 10.1093/nar/gky094.


Xenotopic expression of alternative electron transport enzymes in animal mitochondria and their impact in health and disease.

Camargo AF, Chioda MM, Rodrigues APC, Garcia GS, McKinney EA, Jacobs HT, Oliveira MT.

Cell Biol Int. 2018 Jun;42(6):664-669. doi: 10.1002/cbin.10943. Epub 2018 Feb 23. Review.


Mitochondria are physiologically maintained at close to 50 °C.

Chrétien D, Bénit P, Ha HH, Keipert S, El-Khoury R, Chang YT, Jastroch M, Jacobs HT, Rustin P, Rak M.

PLoS Biol. 2018 Jan 25;16(1):e2003992. doi: 10.1371/journal.pbio.2003992. eCollection 2018 Jan.


Intracellular vesicle trafficking plays an essential role in mitochondrial quality control.

Gerards M, Cannino G, de Cózar JMG, Jacobs HT.

Mol Biol Cell. 2018 Jan 17. pii: mbc.E17-10-0619. doi: 10.1091/mbc.E17-10-0619. [Epub ahead of print]


Expression of Ciona intestinalis AOX causes male reproductive defects in Drosophila melanogaster.

Saari S, Andjelković A, Garcia GS, Jacobs HT, Oliveira MT.

BMC Dev Biol. 2017 Jul 3;17(1):9. doi: 10.1186/s12861-017-0151-3.


Mitochondrial genotype modulates mtDNA copy number and organismal phenotype in Drosophila.

Salminen TS, Oliveira MT, Cannino G, Lillsunde P, Jacobs HT, Kaguni LS.

Mitochondrion. 2017 May;34:75-83. doi: 10.1016/j.mito.2017.02.001. Epub 2017 Feb 16.


Broad AOX expression in a genetically tractable mouse model does not disturb normal physiology.

Szibor M, Dhandapani PK, Dufour E, Holmström KM, Zhuang Y, Salwig I, Wittig I, Heidler J, Gizatullina Z, Gainutdinov T; German Mouse Clinic Consortium, Fuchs H, Gailus-Durner V, de Angelis MH, Nandania J, Velagapudi V, Wietelmann A, Rustin P, Gellerich FN, Jacobs HT, Braun T.

Dis Model Mech. 2017 Feb 1;10(2):163-171. doi: 10.1242/dmm.027839. Epub 2016 Dec 14.


Succinate Dehydrogenase Supports Metabolic Repurposing of Mitochondria to Drive Inflammatory Macrophages.

Mills EL, Kelly B, Logan A, Costa ASH, Varma M, Bryant CE, Tourlomousis P, Däbritz JHM, Gottlieb E, Latorre I, Corr SC, McManus G, Ryan D, Jacobs HT, Szibor M, Xavier RJ, Braun T, Frezza C, Murphy MP, O'Neill LA.

Cell. 2016 Oct 6;167(2):457-470.e13. doi: 10.1016/j.cell.2016.08.064. Epub 2016 Sep 22.


Ligand-Bound GeneSwitch Causes Developmental Aberrations in Drosophila that Are Alleviated by the Alternative Oxidase.

Andjelković A, Kemppainen KK, Jacobs HT.

G3 (Bethesda). 2016 Sep 8;6(9):2839-46. doi: 10.1534/g3.116.030882.


Mitochondrial transcription termination factor 1 directs polar replication fork pausing.

Shi Y, Posse V, Zhu X, Hyvärinen AK, Jacobs HT, Falkenberg M, Gustafsson CM.

Nucleic Acids Res. 2016 Jul 8;44(12):5732-42. doi: 10.1093/nar/gkw302. Epub 2016 Apr 25.


Expression of the alternative oxidase mitigates beta-amyloid production and toxicity in model systems.

El-Khoury R, Kaulio E, Lassila KA, Crowther DC, Jacobs HT, Rustin P.

Free Radic Biol Med. 2016 Jul;96:57-66. doi: 10.1016/j.freeradbiomed.2016.04.006. Epub 2016 Apr 14.


Correction: Mitochondrial Dysfunction Plus High-Sugar Diet Provokes a Metabolic Crisis That Inhibits Growth.

Kemppainen E, George J, Garipler G, Tuomela T, Kiviranta E, Soga T, Dunn CD, Jacobs HT.

PLoS One. 2016 Mar 8;11(3):e0151421. doi: 10.1371/journal.pone.0151421. eCollection 2016. No abstract available.


Mitochondrial Dysfunction Plus High-Sugar Diet Provokes a Metabolic Crisis That Inhibits Growth.

Kemppainen E, George J, Garipler G, Tuomela T, Kiviranta E, Soga T, Dunn CD, Jacobs HT.

PLoS One. 2016 Jan 26;11(1):e0145836. doi: 10.1371/journal.pone.0145836. eCollection 2016. Erratum in: PLoS One. 2016;11(3):e0151421.


Diiron centre mutations in Ciona intestinalis alternative oxidase abolish enzymatic activity and prevent rescue of cytochrome oxidase deficiency in flies.

Andjelković A, Oliveira MT, Cannino G, Yalgin C, Dhandapani PK, Dufour E, Rustin P, Szibor M, Jacobs HT.

Sci Rep. 2015 Dec 17;5:18295. doi: 10.1038/srep18295.


β carbonic anhydrase is required for female fertility in Drosophila melanogaster.

Syrjänen L, Valanne S, Kuuslahti M, Tuomela T, Sriram A, Sanz A, Jacobs HT, Rämet M, Parkkila S.

Front Zool. 2015 Aug 22;12:19. doi: 10.1186/s12983-015-0111-3. eCollection 2015.


Primer retention owing to the absence of RNase H1 is catastrophic for mitochondrial DNA replication.

Holmes JB, Akman G, Wood SR, Sakhuja K, Cerritelli SM, Moss C, Bowmaker MR, Jacobs HT, Crouch RJ, Holt IJ.

Proc Natl Acad Sci U S A. 2015 Jul 28;112(30):9334-9. doi: 10.1073/pnas.1503653112. Epub 2015 Jul 10.


A rolling circle replication mechanism produces multimeric lariats of mitochondrial DNA in Caenorhabditis elegans.

Lewis SC, Joers P, Willcox S, Griffith JD, Jacobs HT, Hyman BC.

PLoS Genet. 2015 Feb 18;11(2):e1004985. doi: 10.1371/journal.pgen.1004985. eCollection 2015 Feb.


Unique features of DNA replication in mitochondria: a functional and evolutionary perspective.

Holt IJ, Jacobs HT.

Bioessays. 2014 Nov;36(11):1024-31. doi: 10.1002/bies.201400052. Epub 2014 Sep 12.


The alternative oxidase AOX does not rescue the phenotype of tko25t mutant flies.

Kemppainen KK, Kemppainen E, Jacobs HT.

G3 (Bethesda). 2014 Aug 21;4(10):2013-21. doi: 10.1534/g3.114.013946.


Ciona intestinalis NADH dehydrogenase NDX confers stress-resistance and extended lifespan on Drosophila.

Gospodaryov DV, Lushchak OV, Rovenko BM, Perkhulyn NV, Gerards M, Tuomela T, Jacobs HT.

Biochim Biophys Acta. 2014 Nov;1837(11):1861-1869. doi: 10.1016/j.bbabio.2014.08.001. Epub 2014 Aug 12.


Screen for mitochondrial DNA copy number maintenance genes reveals essential role for ATP synthase.

Fukuoh A, Cannino G, Gerards M, Buckley S, Kazancioglu S, Scialo F, Lihavainen E, Ribeiro A, Dufour E, Jacobs HT.

Mol Syst Biol. 2014 Jun 21;10:734. doi: 10.15252/msb.20145117.


Phenotypic rescue of a Drosophila model of mitochondrial ANT1 disease.

Vartiainen S, Chen S, George J, Tuomela T, Luoto KR, O'Dell KM, Jacobs HT.

Dis Model Mech. 2014 Jun;7(6):635-48. doi: 10.1242/dmm.016527. Epub 2014 May 8.


Engineering the alternative oxidase gene to better understand and counteract mitochondrial defects: state of the art and perspectives.

El-Khoury R, Kemppainen KK, Dufour E, Szibor M, Jacobs HT, Rustin P.

Br J Pharmacol. 2014 Apr;171(8):2243-9. doi: 10.1111/bph.12570. Review.


Expression of alternative oxidase in Drosophila ameliorates diverse phenotypes due to cytochrome oxidase deficiency.

Kemppainen KK, Rinne J, Sriram A, Lakanmaa M, Zeb A, Tuomela T, Popplestone A, Singh S, Sanz A, Rustin P, Jacobs HT.

Hum Mol Genet. 2014 Apr 15;23(8):2078-93. doi: 10.1093/hmg/ddt601. Epub 2013 Nov 29.


Mitochondrial transcription terminator family members mTTF and mTerf5 have opposing roles in coordination of mtDNA synthesis.

Jõers P, Lewis SC, Fukuoh A, Parhiala M, Ellilä S, Holt IJ, Jacobs HT.

PLoS Genet. 2013;9(9):e1003800. doi: 10.1371/journal.pgen.1003800. Epub 2013 Sep 19.


Mitochondrial DNA replication proceeds via a 'bootlace' mechanism involving the incorporation of processed transcripts.

Reyes A, Kazak L, Wood SR, Yasukawa T, Jacobs HT, Holt IJ.

Nucleic Acids Res. 2013 Jun;41(11):5837-50. doi: 10.1093/nar/gkt196. Epub 2013 Apr 16.


A mitochondrial ribosomal and RNA decay pathway blocks cell proliferation.

Richter U, Lahtinen T, Marttinen P, Myöhänen M, Greco D, Cannino G, Jacobs HT, Lietzén N, Nyman TA, Battersby BJ.

Curr Biol. 2013 Mar 18;23(6):535-41. doi: 10.1016/j.cub.2013.02.019. Epub 2013 Feb 28.


Alternative oxidase expression in the mouse enables bypassing cytochrome c oxidase blockade and limits mitochondrial ROS overproduction.

El-Khoury R, Dufour E, Rak M, Ramanantsoa N, Grandchamp N, Csaba Z, Duvillié B, Bénit P, Gallego J, Gressens P, Sarkis C, Jacobs HT, Rustin P.

PLoS Genet. 2013;9(1):e1003182. doi: 10.1371/journal.pgen.1003182. Epub 2013 Jan 3.


Glucose modulates respiratory complex I activity in response to acute mitochondrial dysfunction.

Cannino G, El-Khoury R, Pirinen M, Hutz B, Rustin P, Jacobs HT, Dufour E.

J Biol Chem. 2012 Nov 9;287(46):38729-40. doi: 10.1074/jbc.M112.386060. Epub 2012 Sep 24.


dj-1β regulates oxidative stress, insulin-like signaling and development in Drosophila melanogaster.

Stefanatos R, Sriram A, Kiviranta E, Mohan A, Ayala V, Jacobs HT, Pamplona R, Sanz A.

Cell Cycle. 2012 Oct 15;11(20):3876-86. doi: 10.4161/cc.22073. Epub 2012 Sep 14.


A cytoplasmic suppressor of a nuclear mutation affecting mitochondrial functions in Drosophila.

Chen S, Oliveira MT, Sanz A, Kemppainen E, Fukuoh A, Schlicht B, Kaguni LS, Jacobs HT.

Genetics. 2012 Oct;192(2):483-93. doi: 10.1534/genetics.112.143719. Epub 2012 Jul 30.


Therapies in inborn errors of oxidative metabolism.

Schiff M, Bénit P, Jacobs HT, Vockley J, Rustin P.

Trends Endocrinol Metab. 2012 Sep;23(9):488-95. doi: 10.1016/j.tem.2012.04.006. Epub 2012 May 25. Review.


Alternative oxidase rescues mitochondria-mediated dopaminergic cell loss in Drosophila.

Humphrey DM, Parsons RB, Ludlow ZN, Riemensperger T, Esposito G, Verstreken P, Jacobs HT, Birman S, Hirth F.

Hum Mol Genet. 2012 Jun 15;21(12):2698-712. doi: 10.1093/hmg/dds096. Epub 2012 Mar 7.


Human mitochondrial transcription factor A induces a U-turn structure in the light strand promoter.

Rubio-Cosials A, Sidow JF, Jiménez-Menéndez N, Fernández-Millán P, Montoya J, Jacobs HT, Coll M, Bernadó P, Solà M.

Nat Struct Mol Biol. 2011 Oct 30;18(11):1281-9. doi: 10.1038/nsmb.2160. Erratum in: Nat Struct Mol Biol. 2012 Mar;19(3):364.


Genome wide study of NF-Y type CCAAT boxes in unidirectional and bidirectional promoters in human and mouse.

Häkkinen A, Healy S, Jacobs HT, Ribeiro AS.

J Theor Biol. 2011 Jul 21;281(1):74-83. doi: 10.1016/j.jtbi.2011.04.027. Epub 2011 May 5.


Effects on mitochondrial transcription of manipulating mTERF protein levels in cultured human HEK293 cells.

Hyvärinen AK, Kumanto MK, Marjavaara SK, Jacobs HT.

BMC Mol Biol. 2010 Sep 16;11:72. doi: 10.1186/1471-2199-11-72.


Stabilization of hypoxia-inducible factor-1alpha protein in hypoxia occurs independently of mitochondrial reactive oxygen species production.

Chua YL, Dufour E, Dassa EP, Rustin P, Jacobs HT, Taylor CT, Hagen T.

J Biol Chem. 2010 Oct 8;285(41):31277-84. doi: 10.1074/jbc.M110.158485. Epub 2010 Jul 30.


Overexpression of MTERFD1 or MTERFD3 impairs the completion of mitochondrial DNA replication.

Hyvärinen AK, Pohjoismäki JL, Holt IJ, Jacobs HT.

Mol Biol Rep. 2011 Feb;38(2):1321-8. doi: 10.1007/s11033-010-0233-9. Epub 2010 Jun 25.


Human mitochondrial mTERF wraps around DNA through a left-handed superhelical tandem repeat.

Jiménez-Menéndez N, Fernández-Millán P, Rubio-Cosials A, Arnan C, Montoya J, Jacobs HT, Bernadó P, Coll M, Usón I, Solà M.

Nat Struct Mol Biol. 2010 Jul;17(7):891-3. doi: 10.1038/nsmb.1859. Epub 2010 Jun 13.


Developmental and pathological changes in the human cardiac muscle mitochondrial DNA organization, replication and copy number.

Pohjoismäki JL, Goffart S, Taylor RW, Turnbull DM, Suomalainen A, Jacobs HT, Karhunen PJ.

PLoS One. 2010 May 3;5(5):e10426. doi: 10.1371/journal.pone.0010426.

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