Publications Research Group Stewart

Extreme heterogeneity of human mitochondrial DNA from organelles to populations

  • Stewart, James B.
  • Chinnery, Patrick F.
(2020) Nature Reviews Genetics

Modulation of mtDNA copy number ameliorates the pathological consequences of a heteroplasmic mtDNA mutation in the mouse

  • Filograna, R.
  • Koolmeister, C.
  • Upadhyay, M.
  • Pajak, A.
  • Clemente, P.
  • Wibom, R.
  • Simard, M. L.
  • Wredenberg, A.
  • Freyer, C.
  • Stewart, J. B.
  • Larsson, N. G.
(2019) Sci Adv, 5, 4, eaav9824

Mitochondrial stress response triggered by defects in protein synthesis quality control

  • Richter, U.
  • Ng, K. Y.
  • Suomi, F.
  • Marttinen, P.
  • Turunen, T.
  • Jackson, C.
  • Suomalainen, A.
  • Vihinen, H.
  • Jokitalo, E.
  • Nyman, T. A.
  • Isokallio, M. A.
  • Stewart, J. B.
  • Mancini, C.
  • Brusco, A.
  • Seneca, S.
  • Lombes, A.
  • Taylor, R. W.
  • Battersby, B. J.
(2019) Life Sci Alliance, 2, 1

Genome editing in mitochondria corrects a pathogenic mtDNA mutation in vivo

  • Gammage, P. A.
  • Viscomi, C.
  • Simard, M. L.
  • Costa, A. S. H.
  • Gaude, E.
  • Powell, C. A.
  • Van Haute, L.
  • McCann, B. J.
  • Rebelo-Guiomar, P.
  • Cerutti, R.
  • Zhang, L.
  • Rebar, E. J.
  • Zeviani, M.
  • Frezza, C.
  • Stewart, J. B.
  • Minczuk, M.
(2018) Nat Med, 24, 11, 1691-1695

MitoTALEN reduces mutant mtDNA load and restores tRNA(Ala) levels in a mouse model of heteroplasmic mtDNA mutation

  • Bacman, S. R.
  • Kauppila, J. H. K.
  • Pereira, C. V.
  • Nissanka, N.
  • Miranda, M.
  • Pinto, M.
  • Williams, S. L.
  • Larsson, N. G.
  • Stewart, J. B.
  • Moraes, C. T.
(2018) Nat Med, 24, 11, 1696-1700

Base-excision repair deficiency alone or combined with increased oxidative stress does not increase mtDNA point mutations in mice

  • Kauppila, J. H. K.
  • Bonekamp, N. A.
  • Mourier, A.
  • Isokallio, M. A.
  • Just, A.
  • Kauppila, T. E. S.
  • Stewart, J. B.
  • Larsson, N. G.
(2018) Nucleic Acids Res, 46, 13, 6642-6669

A novel histochemistry assay to assess and quantify focal cytochrome c oxidase deficiency

  • Simard, M. L.
  • Mourier, A.
  • Greaves, L. C.
  • Taylor, R. W.
  • Stewart, J. B.**
(2018) J Pathol, 245, 3, 311-323

Mice lacking the mitochondrial exonuclease MGME1 accumulate mtDNA deletions without developing progeria

  • Matic, S.
  • Jiang, M.
  • Nicholls, T. J.
  • Uhler, J. P.
  • Dirksen-Schwanenland, C.
  • Polosa, P. L.
  • Simard, M. L.
  • Li, X.
  • Atanassov, I.
  • Rackham, O.
  • Filipovska, A.
  • Stewart, J. B.
  • Falkenberg, M.
  • Larsson, N. G.
  • Milenkovic, D.
(2018) Nat Commun, 9, 1, 1202

Delivery of mtZFNs into Early Mouse Embryos

  • McCann, B. J.
  • Cox, A.
  • Gammage, P. A.
  • Stewart, J. B.
  • Zernicka-Goetz, M.
  • Minczuk, M.
(2018) Methods Mol Biol, 1867, 215-228

Increased Total mtDNA Copy Number Cures Male Infertility Despite Unaltered mtDNA Mutation Load

  • Jiang, M.
  • Kauppila, T. E. S.
  • Motori, E.
  • Li, X.
  • Atanassov, I.
  • Folz-Donahue, K.
  • Bonekamp, N. A.
  • Albarran-Gutierrez, S.
  • Stewart, J. B.
  • Larsson, N. G.
(2017) Cell Metab, 26, 2, 429-436 e4

A Phenotype-Driven Approach to Generate Mouse Models with Pathogenic mtDNA Mutations Causing Mitochondrial Disease

  • Kauppila, J. H.
  • Baines, H. L.
  • Bratic, A.
  • Simard, M. L.
  • Freyer, C.
  • Mourier, A.
  • Stamp, C.
  • Filograna, R.
  • Larsson, N. G.**
  • Greaves, L. C.
  • Stewart, J. B.**
(2016) Cell Rep, 16, 11, 2980-90

Hierarchical RNA Processing Is Required for Mitochondrial Ribosome Assembly

  • Rackham, O.
  • Busch, J. D.
  • Matic, S.
  • Siira, S. J.
  • Kuznetsova, I.
  • Atanassov, I.
  • Ermer, J. A.
  • Shearwood, A. M.
  • Richman, T. R.
  • Stewart, J. B.
  • Mourier, A.
  • Milenkovic, D.
  • Larsson, N. G.
  • Filipovska, A.
(2016) Cell Rep, 16, 7, 1874-90

Tissue-specific modulation of mitochondrial DNA segregation by a defect in mitochondrial division

  • Jokinen, R.
  • Marttinen, P.
  • Stewart, J. B.
  • Neil Dear, T.
  • Battersby, B. J.
(2016) Hum Mol Genet, 25, 4, 706-14

Complementation between polymerase- and exonuclease-deficient mitochondrial DNA polymerase mutants in genomically engineered flies

  • Bratic, A.
  • Kauppila, T. E.
  • Macao, B.
  • Gronke, S.
  • Siibak, T.
  • Stewart, J. B.
  • Baggio, F.
  • Dols, J.
  • Partridge, L.
  • Falkenberg, M.
  • Wredenberg, A.
  • Larsson, N. G.
(2015) Nat Commun, 6, 8808

Mitochondrial DNA: Radically free of free-radical driven mutations

  • Kauppila, J. H.
  • Stewart, J. B.**
(2015) Biochim Biophys Acta, 1847, 11, 1354-61

The dynamics of mitochondrial DNA heteroplasmy: implications for human health and disease

  • Stewart, J. B.
  • Chinnery, P. F.
(2015) Nat Rev Genet, 16, 9, 530-42

The exonuclease activity of DNA polymerase gamma is required for ligation during mitochondrial DNA replication

  • Macao, B.
  • Uhler, J. P.
  • Siibak, T.
  • Zhu, X.
  • Shi, Y.
  • Sheng, W.
  • Olsson, M.
  • Stewart, J. B.
  • Gustafsson, C. M.
  • Falkenberg, M.
(2015) Nat Commun, 6, 7303

Simultaneous DNA and RNA Mapping of Somatic Mitochondrial Mutations across Diverse Human Cancers

  • Stewart, J. B.
  • Alaei-Mahabadi, B.
  • Sabarinathan, R.
  • Samuelsson, T.
  • Gorodkin, J.
  • Gustafsson, C. M.
  • Larsson, E.
(2015) PLoS Genet, 11, 6, e1005333

Keeping mtDNA in shape between generations

  • Stewart, J. B.**
  • Larsson, N. G.**
(2014) PLoS Genet, 10, 10, e1004670

Similar patterns of clonally expanded somatic mtDNA mutations in the colon of heterozygous mtDNA mutator mice and ageing humans

  • Baines, H. L.
  • Stewart, J. B.
  • Stamp, C.
  • Zupanic, A.
  • Kirkwood, T. B.
  • Larsson, N. G.
  • Turnbull, D. M.
  • Greaves, L. C.
(2014) Mech Ageing Dev, 139, 22-30

The challenges of mitochondrial replacement

  • Chinnery, P. F.
  • Craven, L.
  • Mitalipov, S.
  • Stewart, J. B.
  • Herbert, M.
  • Turnbull, D. M.
(2014) PLoS Genet, 10, 4, e1004315

No recombination of mtDNA after heteroplasmy for 50 generations in the mouse maternal germline

  • Hagstrom, E.
  • Freyer, C.
  • Battersby, B. J.
  • Stewart, J. B.**
  • Larsson, N. G.**
(2014) Nucleic Acids Res, 42, 2, 1111-6

Characterization of the sea urchin mitochondrial transcription factor A reveals unusual features

  • Deceglie, S.
  • Lionetti, C.
  • Stewart, J. B.
  • Habermann, B.
  • Roberti, M.
  • Cantatore, P.
  • Loguercio Polosa, P.
(2014) Mitochondrion, 14, 1, 34-41

Addressing RNA integrity to determine the impact of mitochondrial DNA mutations on brain mitochondrial function with age

  • Wang, W.
  • Scheffler, K.
  • Esbensen, Y.
  • Strand, J. M.
  • Stewart, J. B.
  • Bjoras, M.
  • Eide, L.
(2014) PLoS One, 9, 5, e96940

Germline mitochondrial DNA mutations aggravate ageing and can impair brain development

  • Ross, J. M.*
  • Stewart, J. B.*
  • Hagstrom, E.
  • Brene, S.
  • Mourier, A.
  • Coppotelli, G.
  • Freyer, C.
  • Lagouge, M.
  • Hoffer, B. J.
  • Olson, L.
  • Larsson, N. G.
(2013) Nature, 501, 7467, 412-5

MTERF1 binds mtDNA to prevent transcriptional interference at the light-strand promoter but is dispensable for rRNA gene transcription regulation

  • Terzioglu, M.
  • Ruzzenente, B.
  • Harmel, J.
  • Mourier, A.
  • Jemt, E.
  • Lopez, M. D.
  • Kukat, C.
  • Stewart, J. B.
  • Wibom, R.
  • Meharg, C.
  • Habermann, B.
  • Falkenberg, M.
  • Gustafsson, C. M.
  • Park, C. B.
  • Larsson, N. G.
(2013) Cell Metab, 17, 4, 618-26

In vivo mutagenesis reveals that OriL is essential for mitochondrial DNA replication

  • Wanrooij, S.
  • Miralles Fuste, J.
  • Stewart, J. B.
  • Wanrooij, P. H.
  • Samuelsson, T.
  • Larsson, N. G.
  • Gustafsson, C. M.
  • Falkenberg, M.
(2012) EMBO Rep, 13, 12, 1130-7

Variation in germline mtDNA heteroplasmy is determined prenatally but modified during subsequent transmission

  • Freyer, C.
  • Cree, L. M.
  • Mourier, A.
  • Stewart, J. B.
  • Koolmeister, C.
  • Milenkovic, D.
  • Wai, T.
  • Floros, V. I.
  • Hagstrom, E.
  • Chatzidaki, E. E.
  • Wiesner, R. J.
  • Samuels, D. C.
  • Larsson, N. G.
  • Chinnery, P. F.
(2012) Nat Genet, 44, 11, 1282-5

Mitochondrial DNA deletions are associated with non-B DNA conformations

  • Damas, J.
  • Carneiro, J.
  • Goncalves, J.
  • Stewart, J. B.
  • Samuels, D. C.
  • Amorim, A.
  • Pereira, F.
(2012) Nucleic Acids Res, 40, 16, 7606-21

LRPPRC is necessary for polyadenylation and coordination of translation of mitochondrial mRNAs

  • Ruzzenente, B.
  • Metodiev, M. D.
  • Wredenberg, A.
  • Bratic, A.
  • Park, C. B.
  • Camara, Y.
  • Milenkovic, D.
  • Zickermann, V.
  • Wibom, R.
  • Hultenby, K.
  • Erdjument-Bromage, H.
  • Tempst, P.
  • Brandt, U.
  • Stewart, J. B.
  • Gustafsson, C. M.
  • Larsson, N. G.
(2012) EMBO J, 31, 2, 443-56

The bicoid stability factor controls polyadenylation and expression of specific mitochondrial mRNAs in Drosophila melanogaster

  • Bratic, A.
  • Wredenberg, A.
  • Gronke, S.
  • Stewart, J. B.
  • Mourier, A.
  • Ruzzenente, B.
  • Kukat, C.
  • Wibom, R.
  • Habermann, B.
  • Partridge, L.
  • Larsson, N. G.
(2011) PLoS Genet, 7, 10, e1002324

Ultra-deep sequencing of mouse mitochondrial DNA: mutational patterns and their origins

  • Ameur, A.*
  • Stewart, J. B.*
  • Freyer, C.
  • Hagstrom, E.
  • Ingman, M.
  • Larsson, N. G.
  • Gyllensten, U.
(2011) PLoS Genet, 7, 3, e1002028

Characterization of mature mitochondrial transcripts in Drosophila, and the implications for the tRNA punctuation model in arthropods

  • Stewart, J. B.**
  • Beckenbach, A. T.
(2009) Gene, 445, 1-2, 49-57

Insect mitochondrial genomics 3: the complete mitochondrial genome sequences of representatives from two neuropteroid orders: a dobsonfly (order Megaloptera) and a giant lacewing and an owlfly (order Neuroptera)

  • Beckenbach, A. T.
  • Stewart, J. B.
(2009) Genome, 52, 1, 31-8

Purifying selection of mtDNA and its implications for understanding evolution and mitochondrial disease

  • Stewart, J. B.
  • Freyer, C.
  • Elson, J. L.
  • Larsson, N. G.
(2008) Nat Rev Genet, 9, 9, 657-62

Progressive loss of mitochondrial DNA in thymidine kinase 2-deficient mice

  • Zhou, X.
  • Solaroli, N.
  • Bjerke, M.
  • Stewart, J. B.
  • Rozell, B.
  • Johansson, M.
  • Karlsson, A.
(2008) Hum Mol Genet, 17, 15, 2329-35

Strong purifying selection in transmission of mammalian mitochondrial DNA

  • Stewart, J. B.**
  • Freyer, C.
  • Elson, J. L.
  • Wredenberg, A.
  • Cansu, Z.
  • Trifunovic, A.
  • Larsson, N. G.
(2008) PLoS Biol, 6, 1, e10

Insect mitochondrial genomics 2: The complete mitochondrial genome sequence of a giant stonefly, Pteronarcys princeps, asymmetric directional mutation bias, and conserved plecopteran A+T-region elements

  • Stewart, J. B.**
  • Beckenbach, A. T.
(2006) Genome, 49, 7, 815-24

Incorporating Molecular Evolution into Phylogenetic Analysis, and a New Compilation of Conserved Polymerase Chain Reaction Primers for Animal Mitochondrial DNA

  • Simon, C.
  • Buckley, T.
  • Frati, F.
  • Stewart, J. B.
  • Beckenbach, A. T.
(2006) Annual Review of Ecology Evolution and Systematics, 37: 545-579

Insect mitochondrial genomics: the complete mitochondrial genome sequence of the meadow spittlebug Philaenus spumarius (Hemiptera: Auchenorrhyncha: Cercopoidae)

  • Stewart, J. B.**
  • Beckenbach, A. T.
(2005) Genome, 48, 1, 46-54

Phylogenetic and genomic analysis of the complete mitochondrial DNA sequence of the spotted asparagus beetle Crioceris duodecimpunctata

  • Stewart, J. B.**
  • Beckenbach, A. T.
(2003) Mol Phylogenet Evol, 26, 3, 513-26

*equal contribution; **corresponding author

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