Saari S, Garcia GS, Bremer K, Chioda MM, Andjelković A, Debes PV, Nikinmaa M, Szibor M, Dufour E, Rustin P, Oliveira MT, Jacobs HT
Alternative respiratory chain enzymes: Therapeutic potential and possible pitfalls.
Biochim Biophys Acta Mol Basis Dis ;1865(4)854-866, 2019

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
Alternative oxidase-mediated respiration prevents lethal mitochondrial cardiomyopathy.
EMBO Mol Med ;2019

González de Cózar JM, Gerards M, Teeri E, George J, Dufour E, Jacobs HT, Jõers P
RNase H1 promotes replication fork progression through oppositely transcribed regions of Drosophila mitochondrial DNA.
J Biol Chem ;2019


Andjelković A, Mordas A, Bruinsma L, Ketola A, Cannino G, Giordano L, Dhandapani PK, Szibor M, Dufour E, Jacobs HT
Expression of the Alternative Oxidase Influences Jun N-Terminal Kinase Signaling and Cell Migration.
Mol Cell Biol ;2018

Dogan SA, Cerutti R, Benincá C, Brea-Calvo G, Jacobs HT, Zeviani M, Szibor M, Viscomi C
Perturbed Redox Signaling Exacerbates a Mitochondrial Myopathy.
Cell Metab ;28(5)764-775.e5, 2018

Saari S, Garcia GS, Bremer K, Chioda MM, Andjelković A, Debes PV, Nikinmaa M, Szibor M, Dufour E, Rustin P, Oliveira MT, Jacobs HT
Alternative respiratory chain enzymes: Therapeutic potential and possible pitfalls.
Biochim Biophys Acta Mol Basis Dis ;2018

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
Alternative Oxidase Attenuates Cigarette Smoke-Induced Lung Dysfunction and Tissue Damage.
Am J Respir Cell Mol Biol ;2018

Rodrigues APC, Camargo AF, Andjelković A, Jacobs HT, Oliveira MT
Developmental arrest in Drosophila melanogaster caused by mitochondrial DNA replication defects cannot be rescued by the alternative oxidase.
Sci Rep ;8(1)10882, 2018

Toompuu M, Tuomela T, Laine P, Paulin L, Dufour E, Jacobs HT
Polyadenylation and degradation of structurally abnormal mitochondrial tRNAs in human cells.
Nucleic Acids Res ;46(10)5209-5226, 2018

Camargo AF, Chioda MM, Rodrigues APC, Garcia GS, McKinney EA, Jacobs HT, Oliveira MT
Xenotopic expression of alternative electron transport enzymes in animal mitochondria and their impact in health and disease.
Cell Biol Int ;42(6)664-669, 2018

Ciesielski GL, Nadalutti CA, Oliveira MT, Jacobs HT, Griffith JD, Kaguni LS
Structural rearrangements in the mitochondrial genome of Drosophila melanogaster induced by elevated levels of the replicative DNA helicase.
Nucleic Acids Res ;46(6)3034-3046, 2018

Chrétien D, Bénit P, Ha HH, Keipert S, El-Khoury R, Chang YT, Jastroch M, Jacobs HT, Rustin P, Rak M
Mitochondria are physiologically maintained at close to 50 °C.
PLoS Biol ;16(1)e2003992, 2018

Gerards M, Cannino G, de Cózar JMG, Jacobs HT
Intracellular vesicle trafficking plays an essential role in mitochondrial quality control.
Mol Biol Cell ;2018


Salminen TS, Oliveira MT, Cannino G, Lillsunde P, Jacobs HT, Kaguni LS
Mitochondrial genotype modulates mtDNA copy number and organismal phenotype in Drosophila.
Mitochondrion ;2017

Saari S, Andjelković A, Garcia GS, Jacobs HT, Oliveira MT
Expression of Ciona intestinalis AOX causes male reproductive defects in Drosophila melanogaster.
BMC Dev Biol ;17(1)9, 2017


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
Succinate Dehydrogenase Supports Metabolic Repurposing of Mitochondria to Drive Inflammatory Macrophages.
Cell ;167(2)457-470.e13, 2016

Andjelković A, Kemppainen KK, Jacobs HT
Ligand-Bound GeneSwitch Causes Developmental Aberrations in Drosophila that Are Alleviated by the Alternative Oxidase.
G3 (Bethesda) ;6(9)2839-46, 2016

Kemppainen E, George J, Garipler G, Tuomela T, Kiviranta E, Soga T, Dunn CD, Jacobs HT
Correction: Mitochondrial Dysfunction Plus High-Sugar Diet Provokes a Metabolic Crisis That Inhibits Growth.
PLoS One ;11(3)e0151421, 2016

Shi Y, Posse V, Zhu X, Hyvärinen AK, Jacobs HT, Falkenberg M, Gustafsson CM
Mitochondrial transcription termination factor 1 directs polar replication fork pausing.
Nucleic Acids Res ;44(12)5732-42, 2016

El-Khoury R, Kaulio E, Lassila KA, Crowther DC, Jacobs HT, Rustin P
Expression of the alternative oxidase mitigates beta-amyloid production and toxicity in model systems.
Free Radic Biol Med ;2016

Kemppainen E, George J, Garipler G, Tuomela T, Kiviranta E, Soga T, Dunn CD, Jacobs HT
Mitochondrial Dysfunction Plus High-Sugar Diet Provokes a Metabolic Crisis That Inhibits Growth.
PLoS One ;11(1)e0145836, 2016


Syrjänen L, Valanne S, Kuuslahti M, Tuomela T, Sriram A, Sanz A, Jacobs HT, Rämet M, Parkkila S
β carbonic anhydrase is required for female fertility in Drosophila melanogaster.
Front Zool ;2015

Holmes JB, Akman G, Wood SR, Sakhuja K, Cerritelli SM, Moss C, Bowmaker MR, Jacobs HT, Crouch RJ, Holt IJ
Primer retention owing to the absence of RNase H1 is catastrophic for mitochondrial DNA replication.
Proc Natl Acad Sci U S A ;112(30)9334-9, 2015

Andjelković A, Oliveira MT, Cannino G, Yalgin C, Dhandapani PK, Dufour E, Rustin P, Szibor M, Jacobs HT
Diiron centre mutations in Ciona intestinalis alternative oxidase abolish enzymatic activity and prevent rescue of cytochrome oxidase deficiency in flies.
Sci Rep ;2015

Lewis SC, Joers P, Willcox S, Griffith JD, Jacobs HT, Hyman BC
A rolling circle replication mechanism produces multimeric lariats of mitochondrial DNA in Caenorhabditis elegans.
PLoS Genet ;11(2)e1004985, 2015


Gospodaryov DV, Lushchak OV, Rovenko BM, Perkhulyn NV, Gerards M, Tuomela T, Jacobs HT
Ciona intestinalis NADH dehydrogenase NDX confers stress-resistance and extended lifespan on Drosophila.
Biochim Biophys Acta ;1837(11)1861-1869, 2014

Holt IJ, Jacobs HT
Unique features of DNA replication in mitochondria: a functional and evolutionary perspective.
Bioessays ;36(11)1024-31, 2014

Kemppainen KK, Kemppainen E, Jacobs HT
The alternative oxidase AOX does not rescue the phenotype of tko25t mutant flies.
G3 (Bethesda) ;4(10)2013-21, 2014

Fukuoh A, Cannino G, Gerards M, Buckley S, Kazancioglu S, Scialo F, Lihavainen E, Ribeiro A, Dufour E, Jacobs HT
Screen for mitochondrial DNA copy number maintenance genes reveals essential role for ATP synthase.
Mol Syst Biol ;2014

Vartiainen S, Chen S, George J, Tuomela T, Luoto KR, O'Dell KM, Jacobs HT
Phenotypic rescue of a Drosophila model of mitochondrial ANT1 disease.
Dis Model Mech ;7(6)635-48, 2014

El-Khoury R, Kemppainen KK, Dufour E, Szibor M, Jacobs HT, Rustin P
Engineering the alternative oxidase gene to better understand and counteract mitochondrial defects: state of the art and perspectives.
Br J Pharmacol ;171(8)2243-9, 2014

Kemppainen KK, Rinne J, Sriram A, Lakanmaa M, Zeb A, Tuomela T, Popplestone A, Singh S, Sanz A, Rustin P, Jacobs HT
Expression of alternative oxidase in Drosophila ameliorates diverse phenotypes due to cytochrome oxidase deficiency.
Hum Mol Genet ;23(8)2078-93, 2014


Richter U, Lahtinen T, Marttinen P, Myöhänen M, Greco D, Cannino G, Jacobs HT, Lietzén N, Nyman TA, Battersby BJ
A mitochondrial ribosomal and RNA decay pathway blocks cell proliferation.
Curr Biol ;23(6)535-41, 2013

Jõers P, Lewis SC, Fukuoh A, Parhiala M, Ellilä S, Holt IJ, Jacobs HT
Mitochondrial transcription terminator family members mTTF and mTerf5 have opposing roles in coordination of mtDNA synthesis.
PLoS Genet ;9(9)e1003800, 2013

Jõers P, Jacobs HT
Analysis of replication intermediates indicates that Drosophila melanogaster mitochondrial DNA replicates by a strand-coupled theta mechanism.
PLoS One ;8(1)e53249, 2013

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
Alternative oxidase expression in the mouse enables bypassing cytochrome c oxidase blockade and limits mitochondrial ROS overproduction.
PLoS Genet ;9(1)e1003182, 2013

Reyes A, Kazak L, Wood SR, Yasukawa T, Jacobs HT, Holt IJ
Mitochondrial DNA replication proceeds via a 'bootlace' mechanism involving the incorporation of processed transcripts.
Nucleic Acids Res ;41(11)5837-50, 2013


Cannino G, El-Khoury R, Pirinen M, Hutz B, Rustin P, Jacobs HT, Dufour E
Glucose modulates respiratory complex I activity in response to acute mitochondrial dysfunction.
J Biol Chem ;287(46)38729-40, 2012

Stefanatos R, Sriram A, Kiviranta E, Mohan A, Ayala V, Jacobs HT, Pamplona R, Sanz A
dj-1β regulates oxidative stress, insulin-like signaling and development in Drosophila melanogaster.
Cell Cycle ;11(20)3876-86, 2012

Chen S, Oliveira MT, Sanz A, Kemppainen E, Fukuoh A, Schlicht B, Kaguni LS, Jacobs HT
A cytoplasmic suppressor of a nuclear mutation affecting mitochondrial functions in Drosophila.
Genetics ;192(2)483-93, 2012

Schiff M, Bénit P, Jacobs HT, Vockley J, Rustin P
Therapies in inborn errors of oxidative metabolism.
Trends Endocrinol Metab ;23(9)488-95, 2012

Humphrey DM, Parsons RB, Ludlow ZN, Riemensperger T, Esposito G, Verstreken P, Jacobs HT, Birman S, Hirth F
Alternative oxidase rescues mitochondria-mediated dopaminergic cell loss in Drosophila.
Hum Mol Genet ;21(12)2698-712, 2012


Rubio-Cosials A, Sidow JF, Jiménez-Menéndez N, Fernández-Millán P, Montoya J, Jacobs HT, Coll M, Bernadó P, Solà M
Human mitochondrial transcription factor A induces a U-turn structure in the light strand promoter.
Nat Struct Mol Biol ;18(11)1281-9, 2011

Häkkinen A, Healy S, Jacobs HT, Ribeiro AS
Genome wide study of NF-Y type CCAAT boxes in unidirectional and bidirectional promoters in human and mouse.
J Theor Biol ;281(1)74-83, 2011

Hyvärinen AK, Pohjoismäki JL, Holt IJ, Jacobs HT
Overexpression of MTERFD1 or MTERFD3 impairs the completion of mitochondrial DNA replication.
Mol Biol Rep ;38(2)1321-8, 2011


Hyvärinen AK, Kumanto MK, Marjavaara SK, Jacobs HT
Effects on mitochondrial transcription of manipulating mTERF protein levels in cultured human HEK293 cells.
BMC Mol Biol ;2010

Jiménez-Menéndez N, Fernández-Millán P, Rubio-Cosials A, Arnan C, Montoya J, Jacobs HT, Bernadó P, Coll M, Usón I, Solà M
Human mitochondrial mTERF wraps around DNA through a left-handed superhelical tandem repeat.
Nat Struct Mol Biol ;17(7)891-3, 2010

Chua YL, Dufour E, Dassa EP, Rustin P, Jacobs HT, Taylor CT, Hagen T
Stabilization of hypoxia-inducible factor-1alpha protein in hypoxia occurs independently of mitochondrial reactive oxygen species production.
J Biol Chem ;285(41)31277-84, 2010

Pohjoismäki JL, Goffart S, Taylor RW, Turnbull DM, Suomalainen A, Jacobs HT, Karhunen PJ
Developmental and pathological changes in the human cardiac muscle mitochondrial DNA organization, replication and copy number.
PLoS One ;5(5)e10426, 2010

Sanz A, Soikkeli M, Portero-Otín M, Wilson A, Kemppainen E, McIlroy G, Ellilä S, Kemppainen KK, Tuomela T, Lakanmaa M, Kiviranta E, Stefanatos R, Dufour E, Hutz B, Naudí A, Jové M, Zeb A, Vartiainen S, Matsuno-Yagi A, Yagi T, Rustin P, Pamplona R, Jacobs HT
Expression of the yeast NADH dehydrogenase Ndi1 in Drosophila confers increased lifespan independently of dietary restriction.
Proc Natl Acad Sci U S A ;107(20)9105-10, 2010

Sanz A, Fernández-Ayala DJ, Stefanatos RK, Jacobs HT
Mitochondrial ROS production correlates with, but does not directly regulate lifespan in Drosophila.
Aging (Albany NY) ;2(4)200-23, 2010

Pohjoismäki JL, Holmes JB, Wood SR, Yang MY, Yasukawa T, Reyes A, Bailey LJ, Cluett TJ, Goffart S, Willcox S, Rigby RE, Jackson AP, Spelbrink JN, Griffith JD, Crouch RJ, Jacobs HT, Holt IJ
Mammalian mitochondrial DNA replication intermediates are essentially duplex but contain extensive tracts of RNA/DNA hybrid.
J Mol Biol ;397(5)1144-55, 2010

Fernández-Ayala DJ, Chen S, Kemppainen E, O'Dell KM, Jacobs HT
Gene expression in a Drosophila model of mitochondrial disease.
PLoS One ;5(1)e8549, 2010


Dassa EP, Dufour E, Gonçalves S, Paupe V, Hakkaart GA, Jacobs HT, Rustin P
Expression of the alternative oxidase complements cytochrome c oxidase deficiency in human cells.
EMBO Mol Med2009 ;1(1)30-6

Fukuoh A, Ohgaki K, Hatae H, Kuraoka I, Aoki Y, Uchiumi T, Jacobs HT, Kang D
DNA conformation-dependent activities of human mitochondrial RNA polymerase.
Genes Cells2009 ;14(8)1029-42

Kemppainen E, Fernández-Ayala DJ, Galbraith LC, O'Dell KM, Jacobs HT
Phenotypic suppression of the Drosophila mitochondrial disease-like mutant tko(25t) by duplication of the mutant gene in its natural chromosomal context.
Mitochondrion2009 ;9(5)353-63

Pohjoismäki JL, Goffart S, Tyynismaa H, Willcox S, Ide T, Kang D, Suomalainen A, Karhunen PJ, Griffith JD, Holt IJ, Jacobs HT
Human heart mitochondrial DNA is organized in complex catenated networks containing abundant four-way junctions and replication forks.
J Biol Chem2009 ;284(32)21446-57

Rustin P, Jacobs HT
Respiratory chain alternative enzymes as tools to better understand and counteract respiratory chain deficiencies in human cells and animals.
Physiol Plant2009 ;137(4)362-70

Dassa EP, Dufour E, Goncalves S, Jacobs HT, Rustin P
The alternative oxidase, a tool for compensating cytochrome c oxidase deficiency in human cells.
Physiol Plant2009 ;137(4)427-34

Zanotto E, Häkkinen A, Teku G, Shen B, Ribeiro AS, Jacobs HT
NF-Y influences directionality of transcription from the bidirectional Mrps12/Sarsm promoter in both mouse and human cells.
Biochim Biophys Acta2009 ;1789(5)432-42

Fernandez-Ayala DJ, Sanz A, Vartiainen S, Kemppainen KK, Babusiak M, Mustalahti E, Costa R, Tuomela T, Zeviani M, Chung J, O'Dell KM, Rustin P, Jacobs HT
Expression of the Ciona intestinalis alternative oxidase (AOX) in Drosophila complements defects in mitochondrial oxidative phosphorylation.
Cell Metab2009 ;9(5)449-60