Publikationen Larsson Gruppe

Studying Mitochondrial Nucleic Acid Synthesis Utilizing Intact Isolated Mitochondria

  • Misic, J.
  • Milenkovic, D.
(2023) Methods Mol Biol, 2615, 219-228

Cell lineage-specific mitochondrial resilience during mammalian organogenesis

  • Burr, S. P.
  • Klimm, F.
  • Glynos, A.
  • Prater, M.
  • Sendon, P.
  • Nash, P.
  • Powell, C. A.
  • Simard, M. L.
  • Bonekamp, N. A.
  • Charl, J.
  • Diaz, H.
  • Bozhilova, L. V.
  • Nie, Y.
  • Zhang, H.
  • Frison, M.
  • Falkenberg, M.
  • Jones, N.
  • Minczuk, M.
  • Stewart, J. B.
  • Chinnery, P. F.
(2023) Cell

Mammalian RNase H1 directs RNA primer formation for mtDNA replication initiation and is also necessary for mtDNA replication completion

  • Misic, J.
  • Milenkovic, D.
  • Al-Behadili, A.
  • Xie, X.
  • Jiang, M.
  • Jiang, S.
  • Filograna, R.
  • Koolmeister, C.
  • Siira, S. J.
  • Jenninger, L.
  • Filipovska, A.
  • Clausen, A. R.
  • Caporali, L.
  • Valentino, M. L.
  • La Morgia, C.
  • Carelli, V.
  • Nicholls, T. J.
  • Wredenberg, A.
  • Falkenberg, M.
  • Larsson, N. G.
(2022) Nucleic Acids Res, 50, 15, 8749-8766

Mice lacking the mitochondrial exonuclease MGME1 develop inflammatory kidney disease with glomerular dysfunction

  • Milenkovic, D.
  • Sanz-Moreno, A.
  • Calzada-Wack, J.
  • Rathkolb, B.
  • Veronica Amarie, O.
  • Gerlini, R.
  • Aguilar-Pimentel, A.
  • Misic, J.
  • Simard, M. L.
  • Wolf, E.
  • Fuchs, H.
  • Gailus-Durner, V.
  • de Angelis, M. H.
  • Larsson, N. G.
(2022) PLoS Genet, 18, 5, e1010190

Starting the engine of the powerhouse: mitochondrial transcription and beyond

  • Miranda, M.
  • Bonekamp, N. A.
  • Kuhl, I.
(2022) Biol Chem

Metabolic resistance to the inhibition of mitochondrial transcription revealed by CRISPR-Cas9 screen

  • Mennuni, M.
  • Filograna, R.
  • Felser, A.
  • Bonekamp, N. A.
  • Giavalisco, P.
  • Lytovchenko, O.
  • Larsson, N. G.
(2022) EMBO Rep, 23, 1, e53054

A common genetic variant of a mitochondrial RNA processing enzyme predisposes to insulin resistance

  • Rossetti, G.
  • Ermer, J. A.
  • Stentenbach, M.
  • Siira, S. J.
  • Richman, T. R.
  • Milenkovic, D.
  • Perks, K. L.
  • Hughes, L. A.
  • Jamieson, E.
  • Xiafukaiti, G.
  • Ward, N. C.
  • Takahashi, S.
  • Gray, N.
  • Viola, H. M.
  • Hool, L. C.
  • Rackham, O.
  • Filipovska, A.
(2021) Sci Adv, 7(39): eabi7514

High levels of TFAM repress mammalian mitochondrial DNA transcription in vivo

  • Bonekamp, N. A.
  • Jiang, M.
  • Motori, E.
  • Garcia Villegas, R.
  • Koolmeister, C.
  • Atanassov, I.
  • Mesaros, A.
  • Park, C. B.
  • Larsson, N. G.
(2021) Life Sci Alliance, 4, 11, e202101034

Cellular pyrimidine imbalance triggers mitochondrial DNA–dependent innate immunity

  • Sprenger, H.-G.
  • MacVicar, T.
  • Bahat, A.
  • Fiedler, K. U.
  • Hermans, S.
  • Ehrentraut, D.
  • Ried, K.
  • Milenkovic, D.
  • Bonekamp, N.
  • Larsson, N.-G.
  • Nolte, H.
  • Giavalisco, P.
  • Langer, T.
(2021) Nature Metabolism, 3, 5, 636-650

Small-molecule inhibitors of human mitochondrial DNA transcription

  • Bonekamp, N. A.
  • Peter, B.
  • Hillen, H. S.
  • Felser, A.
  • Bergbrede, T.
  • Choidas, A.
  • Horn, M.
  • Unger, A.
  • Di Lucrezia, R.
  • Atanassov, I.
  • Li, X.
  • Koch, U.
  • Menninger, S.
  • Boros, J.
  • Habenberger, P.
  • Giavalisco, P.
  • Cramer, P.
  • Denzel, M. S.
  • Nussbaumer, P.
  • Klebl, B.
  • Falkenberg, M.
  • Gustafsson, C. M.
  • Larsson, N. G.
(2020) Nature, 588, 7839, 712-716

Reweaving the Fabric of Mitochondrial Contact Sites in Astrocytes

  • Bergami, M.
  • Motori, E.
(2020) Front Cell Dev Biol, 8: 592651

DARS2 is indispensable for Purkinje cell survival and protects against cerebellar ataxia

  • Rumyantseva, A.
  • Motori, E.
  • Trifunovic, A.
(2020) Hum Mol Genet, 29(17): 2845-2854

Proofreading deficiency in mitochondrial DNA polymerase does not affect total dNTP pools in mouse embryos

  • Sharma, S.
  • Koolmeister, C.
  • Tran, P.
  • Nilsson, A. K.
  • Larsson, N. G.
  • Chabes, A.
(2020) Nat Metab, 2, 8, 673-675

Neuronal metabolic rewiring promotes resilience to neurodegeneration caused by mitochondrial dysfunction

  • Motori, E.
  • Atanassov, I.
  • Kochan, S. M. V.
  • Folz-Donahue, K.
  • Sakthivelu, V.
  • Giavalisco, P.
  • Toni, N.
  • Puyal, J.
  • Larsson, N. G.
(2020) Sci Adv, 6, 35, eaba8271

FBXL4 deficiency increases mitochondrial removal by autophagy

  • Alsina, D.
  • Lytovchenko, O.
  • Schab, A.
  • Atanassov, I.
  • Schober, F. A.
  • Jiang, M.
  • Koolmeister, C.
  • Wedell, A.
  • Taylor, R. W.
  • Wredenberg, A.
  • Larsson, N. G.
(2020) EMBO Mol Med, 12, 7, e11659

Mitochondria in human disease

  • Larsson, N. G.
  • Wedell, A.
(2020) J Intern Med, 287, 6, 589-591

Dinucleotide Degradation by REXO2 Maintains Promoter Specificity in Mammalian Mitochondria

  • Nicholls, T. J.
  • Spahr, H.
  • Jiang, S.
  • Siira, S. J.
  • Koolmeister, C.
  • Sharma, S.
  • Kauppila, J. H. K.
  • Jiang, M.
  • Kaever, V.
  • Rackham, O.
  • Chabes, A.
  • Falkenberg, M.
  • Filipovska, A.
  • Larsson, N. G.
  • Gustafsson, C. M.
(2019) Mol Cell, 76, 5, 784-796 e6

MitoRibo-Tag Mice Provide a Tool for In Vivo Studies of Mitoribosome Composition

  • Busch, J. D.
  • Cipullo, M.
  • Atanassov, I.
  • Bratic, A.
  • Silva Ramos, E.
  • Schondorf, T.
  • Li, X.
  • Pearce, S. F.
  • Milenkovic, D.
  • Rorbach, J.
  • Larsson, N. G.
(2019) Cell Rep, 29, 6, 1728-1738 e9

Pleiotropic Mitochondria: The Influence of Mitochondria on Neuronal Development and Disease

  • Rangaraju, V.
  • Lewis, T. L., Jr.
  • Hirabayashi, Y.
  • Bergami, M.
  • Motori, E.
  • Cartoni, R.
  • Kwon, S. K.
  • Courchet, J.
(2019) J Neurosci, 39(42): 8200-8208

Mitochondrial fusion is required for regulation of mitochondrial DNA replication

  • Silva Ramos, E.
  • Motori, E.
  • Bruser, C.
  • Kuhl, I.
  • Yeroslaviz, A.
  • Ruzzenente, B.
  • Kauppila, J. H. K.
  • Busch, J. D.
  • Hultenby, K.
  • Habermann, B. H.
  • Jakobs, S.
  • Larsson, N. G.
  • Mourier, A.
(2019) PLoS Genet, 15, 6, e1008085

TEFM regulates both transcription elongation and RNA processing in mitochondria

  • Jiang, S.
  • Koolmeister, C.
  • Misic, J.
  • Siira, S.
  • Kuhl, I.
  • Silva Ramos, E.
  • Miranda, M.
  • Jiang, M.
  • Posse, V.
  • Lytovchenko, O.
  • Atanassov, I.
  • Schober, F. A.
  • Wibom, R.
  • Hultenby, K.
  • Milenkovic, D.
  • Gustafsson, C. M.
  • Filipovska, A.
  • Larsson, N. G.
(2019) EMBO Rep, 20, 6

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

Author Correction: 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, 12, 1940

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

Mutations of mitochondrial DNA are not major contributors to aging of fruit flies

  • Kauppila, T. E. S.
  • Bratic, A.
  • Jensen, M. B.
  • Baggio, F.
  • Partridge, L.
  • Jasper, H.
  • Gronke, S.
  • Larsson, N. G.
(2018) Proc Natl Acad Sci U S A, 115, 41, E9620-E9629

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

Spatiotemporal control of mitochondrial network dynamics in astroglial cells

  • Göbel, J.
  • Motori, E.
  • Bergami, M.
(2018) Biochem Biophys Res Commun, 500(1): 17-25

PTCD1 Is Required for 16S rRNA Maturation Complex Stability and Mitochondrial Ribosome Assembly

  • Perks, K. L.
  • Rossetti, G.
  • Kuznetsova, I.
  • Hughes, L. A.
  • Ermer, J. A.
  • Ferreira, N.
  • Busch, J. D.
  • Rudler, D. L.
  • Spahr, H.
  • Schondorf, T.
  • Shearwood, A. J.
  • Viola, H. M.
  • Siira, S. J.
  • Hool, L. C.
  • Milenkovic, D.
  • Larsson, N. G.
  • Rackham, O.
  • Filipovska, A.
(2018) Cell Rep, 23, 1, 127-142

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

SnapShot: Mitochondrial Nucleoid

  • Bonekamp, N. A.
  • Larsson, N. G.
(2018) Cell, 172, 1-2, 388-388 e1

Topoisomerase 3alpha Is Required for Decatenation and Segregation of Human mtDNA

  • Nicholls, T. J.
  • Nadalutti, C. A.
  • Motori, E.
  • Sommerville, E. W.
  • Gorman, G. S.
  • Basu, S.
  • Hoberg, E.
  • Turnbull, D. M.
  • Chinnery, P. F.
  • Larsson, N. G.
  • Larsson, E.
  • Falkenberg, M.
  • Taylor, R. W.
  • Griffith, J. D.
  • Gustafsson, C. M.
(2018) Mol Cell, 69, 1, 9-23 e6

LRPPRC-mediated folding of the mitochondrial transcriptome

  • Siira, S. J.
  • Spahr, H.
  • Shearwood, A. J.
  • Ruzzenente, B.
  • Larsson, N. G.
  • Rackham, O.
  • Filipovska, A.
(2017) Nat Commun, 8, 1, 1532

Transcriptomic and proteomic landscape of mitochondrial dysfunction reveals secondary coenzyme Q deficiency in mammals

  • Kuhl, I.
  • Miranda, M.
  • Atanassov, I.
  • Kuznetsova, I.
  • Hinze, Y.
  • Mourier, A.
  • Filipovska, A.
  • Larsson, N. G.
(2017) Elife, 6

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

Changes of mitochondrial ultrastructure and function during ageing in mice and Drosophila

  • Brandt, T.
  • Mourier, A.
  • Tain, L. S.
  • Partridge, L.
  • Larsson, N. G.
  • Kuhlbrandt, W.
(2017) Elife, 6

A multi-systemic mitochondrial disorder due to a dominant p.Y955H disease variant in DNA polymerase gamma

  • Siibak, T.
  • Clemente, P.
  • Bratic, A.
  • Bruhn, H.
  • Kauppila, T. E. S.
  • Macao, B.
  • Schober, F. A.
  • Lesko, N.
  • Wibom, R.
  • Naess, K.
  • Nennesmo, I.
  • Wedell, A.
  • Peter, B.
  • Freyer, C.
  • Falkenberg, M.
  • Wredenberg, A.
(2017) Hum Mol Genet, 26, 13, 2515-2525

The Enigma of the Respiratory Chain Supercomplex

  • Milenkovic, D.
  • Blaza, J. N.
  • Larsson, N. G.
  • Hirst, J.
(2017) Cell Metab, 25, 4, 765-776

An Adaptable High-Throughput Technology Enabling the Identification of Specific Transcription Modulators

  • Bergbrede, T.
  • Hoberg, E.
  • Larsson, N. G.
  • Falkenberg, M.
  • Gustafsson, C. M.
(2017) SLAS Discov, 22, 4, 378-386

Mammalian Mitochondria and Aging: An Update

  • Kauppila, T. E.
  • Kauppila, J. H.
  • Larsson, N. G.
(2017) Cell Metab, 25, 1, 57-71

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

COX7A2L Is a Mitochondrial Complex III Binding Protein that Stabilizes the III2+IV Supercomplex without Affecting Respirasome Formation

  • Perez-Perez, R.
  • Lobo-Jarne, T.
  • Milenkovic, D.
  • Mourier, A.
  • Bratic, A.
  • Garcia-Bartolome, A.
  • Fernandez-Vizarra, E.
  • Cadenas, S.
  • Delmiro, A.
  • Garcia-Consuegra, I.
  • Arenas, J.
  • Martin, M. A.
  • Larsson, N. G.
  • Ugalde, C.
(2016) Cell Rep, 16, 9, 2387-98

SLIRP stabilizes LRPPRC via an RRM-PPR protein interface

  • Spahr, H.
  • Rozanska, A.
  • Li, X.
  • Atanassov, I.
  • Lightowlers, R. N.
  • Chrzanowska-Lightowlers, Z. M.
  • Rackham, O.
  • Larsson, N. G.
(2016) Nucleic Acids Res, 44, 14, 6868-82

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

POLRMT regulates the switch between replication primer formation and gene expression of mammalian mtDNA

  • Kuhl, I.
  • Miranda, M.
  • Posse, V.
  • Milenkovic, D.
  • Mourier, A.
  • Siira, S. J.
  • Bonekamp, N. A.
  • Neumann, U.
  • Filipovska, A.
  • Polosa, P. L.
  • Gustafsson, C. M.
  • Larsson, N. G.
(2016) Sci Adv, 2, 8, e1600963

Loss of the RNA-binding protein TACO1 causes late-onset mitochondrial dysfunction in mice

  • Richman, T. R.
  • Spahr, H.
  • Ermer, J. A.
  • Davies, S. M.
  • Viola, H. M.
  • Bates, K. A.
  • Papadimitriou, J.
  • Hool, L. C.
  • Rodger, J.
  • Larsson, N. G.
  • Rackham, O.
  • Filipovska, A.
(2016) Nat Commun, 7, 11884

Maintenance and Expression of Mammalian Mitochondrial DNA

  • Gustafsson, C. M.
  • Falkenberg, M.
  • Larsson, N. G.
(2016) Annu Rev Biochem, 85, 133-60

Bioenergetic roles of mitochondrial fusion

  • Silva Ramos, E.
  • Larsson, N. G.
  • Mourier, A.
(2016) Biochim Biophys Acta, 1857, 8, 1277-83

Overexpression of the mitochondrial methyltransferase TFB1M in the mouse does not impact mitoribosomal methylation status or hearing

  • Lee, S.
  • Rose, S.
  • Metodiev, M. D.
  • Becker, L.
  • Vernaleken, A.
  • Klopstock, T.
  • Gailus-Durner, V.
  • Fuchs, H.
  • Hrabe De Angelis, M.
  • Douthwaite, S.
  • Larsson, N. G.
(2015) Hum Mol Genet, 24, 25, 7286-94

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

Cross-strand binding of TFAM to a single mtDNA molecule forms the mitochondrial nucleoid

  • Kukat, C.
  • Davies, K. M.
  • Wurm, C. A.
  • Spahr, H.
  • Bonekamp, N. A.
  • Kuhl, I.
  • Joos, F.
  • Polosa, P. L.
  • Park, C. B.
  • Posse, V.
  • Falkenberg, M.
  • Jakobs, S.
  • Kuhlbrandt, W.
  • Larsson, N. G.
(2015) Proc Natl Acad Sci U S A, 112, 36, 11288-93

SLIRP Regulates the Rate of Mitochondrial Protein Synthesis and Protects LRPPRC from Degradation

  • Lagouge, M.
  • Mourier, A.
  • Lee, H. J.
  • Spahr, H.
  • Wai, T.
  • Kukat, C.
  • Silva Ramos, E.
  • Motori, E.
  • Busch, J. D.
  • Siira, S.
  • Kremmer, E.
  • Filipovska, A.
  • Larsson, N. G.
(2015) PLoS Genet, 11, 8, e1005423

Mic10 Oligomerization Pinches off Mitochondrial Cristae

  • Milenkovic, D.
  • Larsson, N. G.
(2015) Cell Metab, 21, 5, 660-1

Mitofusin 2 is required to maintain mitochondrial coenzyme Q levels

  • Mourier, A.
  • Motori, E.
  • Brandt, T.
  • Lagouge, M.
  • Atanassov, I.
  • Galinier, A.
  • Rappl, G.
  • Brodesser, S.
  • Hultenby, K.
  • Dieterich, C.
  • Larsson, N. G.
(2015) J Cell Biol, 208, 4, 429-42

Drosophila melanogaster LRPPRC2 is involved in coordination of mitochondrial translation

  • Baggio, F.
  • Bratic, A.
  • Mourier, A.
  • Kauppila, T. E.
  • Tain, L. S.
  • Kukat, C.
  • Habermann, B.
  • Partridge, L.
  • Larsson, N. G.
(2014) Nucleic Acids Res, 42, 22, 13920-38

The respiratory chain supercomplex organization is independent of COX7a2l isoforms

  • Mourier, A.
  • Matic, S.
  • Ruzzenente, B.
  • Larsson, N. G.
  • Milenkovic, D.
(2014) Cell Metab, 20, 6, 1069-75

A human mitochondrial poly(A) polymerase mutation reveals the complexities of post-transcriptional mitochondrial gene expression

  • Wilson, W. C.
  • Hornig-Do, H. T.
  • Bruni, F.
  • Chang, J. H.
  • Jourdain, A. A.
  • Martinou, J. C.
  • Falkenberg, M.
  • Spahr, H.
  • Larsson, N. G.
  • Lewis, R. J.
  • Hewitt, L.
  • Basle, A.
  • Cross, H. E.
  • Tong, L.
  • Lebel, R. R.
  • Crosby, A. H.
  • Chrzanowska-Lightowlers, Z. M.
  • Lightowlers, R. N.
(2014) Hum Mol Genet, 23, 23, 6345-55

Defects in beta-cell Ca2+ dynamics in age-induced diabetes

  • Li, L.
  • Trifunovic, A.
  • Kohler, M.
  • Wang, Y.
  • Petrovic Berglund, J.
  • Illies, C.
  • Juntti-Berggren, L.
  • Larsson, N. G.
  • Berggren, P. O.
(2014) Diabetes, 63, 12, 4100-14

Loss of TFB1M results in mitochondrial dysfunction that leads to impaired insulin secretion and diabetes

  • Sharoyko, V. V.
  • Abels, M.
  • Sun, J.
  • Nicholas, L. M.
  • Mollet, I. G.
  • Stamenkovic, J. A.
  • Gohring, I.
  • Malmgren, S.
  • Storm, P.
  • Fadista, J.
  • Spegel, P.
  • Metodiev, M. D.
  • Larsson, N. G.
  • Eliasson, L.
  • Wierup, N.
  • Mulder, H.
(2014) Hum Mol Genet, 23, 21, 5733-49

POLRMT does not transcribe nuclear genes

  • Kuhl, I.
  • Kukat, C.
  • Ruzzenente, B.
  • Milenkovic, D.
  • Mourier, A.
  • Miranda, M.
  • Koolmeister, C.
  • Falkenberg, M.
  • Larsson, N. G.
(2014) Nature, 514, 7521, E7-11

Adipose tissue mitochondrial dysfunction triggers a lipodystrophic syndrome with insulin resistance, hepatosteatosis, and cardiovascular complications

  • Vernochet, C.
  • Damilano, F.
  • Mourier, A.
  • Bezy, O.
  • Mori, M. A.
  • Smyth, G.
  • Rosenzweig, A.
  • Larsson, N. G.
  • Kahn, C. R.
(2014) Faseb j, 28, 10, 4408-19

Keeping mtDNA in shape between generations

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

Making proteins in the powerhouse

  • Hallberg, B. M.
  • Larsson, N. G.
(2014) Cell Metab, 20, 2, 226-40

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

Loss of LRPPRC causes ATP synthase deficiency

  • Mourier, A.
  • Ruzzenente, B.
  • Brandt, T.
  • Kuhlbrandt, W.
  • Larsson, N. G.
(2014) Hum Mol Genet, 23, 10, 2580-92

The amino terminal extension of mammalian mitochondrial RNA polymerase ensures promoter specific transcription initiation

  • Posse, V.
  • Hoberg, E.
  • Dierckx, A.
  • Shahzad, S.
  • Koolmeister, C.
  • Larsson, N. G.
  • Wilhelmsson, L. M.
  • Hallberg, B. M.
  • Gustafsson, C. M.
(2014) Nucleic Acids Res, 42, 6, 3638-47

NSUN4 is a dual function mitochondrial protein required for both methylation of 12S rRNA and coordination of mitoribosomal assembly

  • Metodiev, M. D.
  • Spahr, H.
  • Loguercio Polosa, P.
  • Meharg, C.
  • Becker, C.
  • Altmueller, J.
  • Habermann, B.
  • Larsson, N. G.
  • Ruzzenente, B.
(2014) PLoS Genet, 10, 2, e1004110

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

The UbL protein UBTD1 stably interacts with the UBE2D family of E2 ubiquitin conjugating enzymes

  • Uhler, J. P.
  • Spahr, H.
  • Farge, G.
  • Clavel, S.
  • Larsson, N. G.
  • Falkenberg, M.
  • Samuelsson, T.
  • Gustafsson, C. M.
(2014) Biochem Biophys Res Commun, 443, 1, 7-12

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

mtDNA makes a U-turn for the mitochondrial nucleoid

  • Kukat, C.
  • Larsson, N. G.
(2013) Trends Cell Biol, 23, 9, 457-63

The role of mitochondrial DNA mutations and free radicals in disease and ageing

  • Lagouge, M.
  • Larsson, N. G.
(2013) J Intern Med, 273, 6, 529-43

The leucine-rich pentatricopeptide repeat-containing protein (LRPPRC) does not activate transcription in mammalian mitochondria

  • Harmel, J.
  • Ruzzenente, B.
  • Terzioglu, M.
  • Spahr, H.
  • Falkenberg, M.
  • Larsson, N. G.
(2013) J Biol Chem, 288, 22, 15510-9

TWINKLE is an essential mitochondrial helicase required for synthesis of nascent D-loop strands and complete mtDNA replication

  • Milenkovic, D.
  • Matic, S.
  • Kuhl, I.
  • Ruzzenente, B.
  • Freyer, C.
  • Jemt, E.
  • Park, C. B.
  • Falkenberg, M.
  • Larsson, N. G.
(2013) Hum Mol Genet, 22, 10, 1983-93

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

The role of mitochondria in aging

  • Bratic, A.
  • Larsson, N. G.
(2013) J Clin Invest, 123, 3, 951-7

MTERF3 regulates mitochondrial ribosome biogenesis in invertebrates and mammals

  • Wredenberg, A.
  • Lagouge, M.
  • Bratic, A.
  • Metodiev, M. D.
  • Spahr, H.
  • Mourier, A.
  • Freyer, C.
  • Ruzzenente, B.
  • Tain, L.
  • Gronke, S.
  • Baggio, F.
  • Kukat, C.
  • Kremmer, E.
  • Wibom, R.
  • Polosa, P. L.
  • Habermann, B.
  • Partridge, L.
  • Park, C. B.
  • Larsson, N. G.
(2013) PLoS Genet, 9, 1, e1003178

Adipose-specific deletion of TFAM increases mitochondrial oxidation and protects mice against obesity and insulin resistance

  • Vernochet, C.
  • Mourier, A.
  • Bezy, O.
  • Macotela, Y.
  • Boucher, J.
  • Rardin, M. J.
  • An, D.
  • Lee, K. Y.
  • Ilkayeva, O. R.
  • Zingaretti, C. M.
  • Emanuelli, B.
  • Smyth, G.
  • Cinti, S.
  • Newgard, C. B.
  • Gibson, B. W.
  • Larsson, N. G.
  • Kahn, C. R.
(2012) Cell Metab, 16, 6, 765-76

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

Mitofusin 2 is necessary for striatal axonal projections of midbrain dopamine neurons

  • Lee, S.
  • Sterky, F. H.
  • Mourier, A.
  • Terzioglu, M.
  • Cullheim, S.
  • Olson, L.
  • Larsson, N. G.
(2012) Hum Mol Genet, 21, 22, 4827-35

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

AFG3L2 supports mitochondrial protein synthesis and Purkinje cell survival

  • Almajan, E. R.
  • Richter, R.
  • Paeger, L.
  • Martinelli, P.
  • Barth, E.
  • Decker, T.
  • Larsson, N. G.
  • Kloppenburg, P.
  • Langer, T.
  • Rugarli, E. I.
(2012) J Clin Invest, 122, 11, 4048-58

Mammalian transcription factor A is a core component of the mitochondrial transcription machinery

  • Shi, Y.
  • Dierckx, A.
  • Wanrooij, P. H.
  • Wanrooij, S.
  • Larsson, N. G.
  • Wilhelmsson, L. M.
  • Falkenberg, M.
  • Gustafsson, C. M.
(2012) Proc Natl Acad Sci U S A, 109(41): 16510-5

Structure of the human MTERF4-NSUN4 protein complex that regulates mitochondrial ribosome biogenesis

  • Spahr, H.
  • Habermann, B.
  • Gustafsson, C. M.
  • Larsson, N. G.
  • Hallberg, B. M.
(2012) Proc Natl Acad Sci U S A, 109(38): 15253-8

Complete Deletion of a POLG1 Allele in a Patient with Alpers Syndrome

  • Naess, K.
  • Barbaro, M.
  • Bruhn, H.
  • Wibom, R.
  • Nennesmo, I.
  • von Dobeln, U.
  • Larsson, N. G.
  • Nemeth, A.
  • Lesko, N.
(2012) JIMD Rep, 4: 67-73

Somatic progenitor cell vulnerability to mitochondrial DNA mutagenesis underlies progeroid phenotypes in Polg mutator mice

  • Ahlqvist, K. J.
  • Hamalainen, R. H.
  • Yatsuga, S.
  • Uutela, M.
  • Terzioglu, M.
  • Gotz, A.
  • Forsstrom, S.
  • Salven, P.
  • Angers-Loustau, A.
  • Kopra, O. H.
  • Tyynismaa, H.
  • Larsson, N. G.
  • Wartiovaara, K.
  • Prolla, T.
  • Trifunovic, A.
  • Suomalainen, A.
(2012) Cell Metab, 15(1): 100-9

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

Altered dopamine metabolism and increased vulnerability to MPTP in mice with partial deficiency of mitochondrial complex I in dopamine neurons

  • Sterky, F. H.
  • Hoffman, A. F.
  • Milenkovic, D.
  • Bao, B.
  • Paganelli, A.
  • Edgar, D.
  • Wibom, R.
  • Lupica, C. R.
  • Olson, L.
  • Larsson, N. G.
(2012) Hum Mol Genet, 21(5): 1078-89

TFAM forces mtDNA to make a U-turn

  • Hallberg, B. M.
  • Larsson, N. G.
(2011) Nat Struct Mol Biol, 18(11): 1179-81

Enhanced cardiomyocyte Ca(2+) cycling precedes terminal AV-block in mitochondrial cardiomyopathy Mterf3 KO mice

  • Andersson, D. C.
  • Fauconnier, J.
  • Park, C. B.
  • Zhang, S. J.
  • Thireau, J.
  • Ivarsson, N.
  • Larsson, N. G.
  • Westerblad, H.
(2011) Antioxid Redox Signal, 15(9): 2455-64

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

The mitochondrial electron transport chain is dispensable for proliferation and differentiation of epidermal progenitor cells

  • Baris, O. R.
  • Klose, A.
  • Kloepper, J. E.
  • Weiland, D.
  • Neuhaus, J. F.
  • Schauen, M.
  • Wille, A.
  • Muller, A.
  • Merkwirth, C.
  • Langer, T.
  • Larsson, N. G.
  • Krieg, T.
  • Tobin, D. J.
  • Paus, R.
  • Wiesner, R. J.
(2011) Stem Cells, 29(9): 1459-68

Super-resolution microscopy reveals that mammalian mitochondrial nucleoids have a uniform size and frequently contain a single copy of mtDNA

  • Kukat, C.
  • Wurm, C. A.
  • Spahr, H.
  • Falkenberg, M.
  • Larsson, N. G.
  • Jakobs, S.
(2011) Proc Natl Acad Sci U S A, 108(33): 13534-9

Impaired mitochondrial transport and Parkin-independent degeneration of respiratory chain-deficient dopamine neurons in vivo

  • Sterky, F. H.
  • Lee, S.
  • Wibom, R.
  • Olson, L.
  • Larsson, N. G.
(2011) Proc Natl Acad Sci U S A, 108(31): 12937-42

Tracing the trail of protons through complex I of the mitochondrial respiratory chain

  • Mourier, A.
  • Larsson, N. G.
(2011) PLoS Biol, 9(8): e1001129

Mitochondrial DNA mutations in disease and aging

  • Park, C. B.
  • Larsson, N. G.
(2011) J Cell Biol, 193(5): 809-18

MTERF4 regulates translation by targeting the methyltransferase NSUN4 to the mammalian mitochondrial ribosome

  • Camara, Y.
  • Asin-Cayuela, J.
  • Park, C. B.
  • Metodiev, M. D.
  • Shi, Y.
  • Ruzzenente, B.
  • Kukat, C.
  • Habermann, B.
  • Wibom, R.
  • Hultenby, K.
  • Franz, T.
  • Erdjument-Bromage, H.
  • Tempst, P.
  • Hallberg, B. M.
  • Gustafsson, C. M.
  • Larsson, N. G.
(2011) Cell Metab, 13(5): 527-39

Unraveling the biological roles of reactive oxygen species

  • Murphy, M. P.
  • Holmgren, A.
  • Larsson, N. G.
  • Halliwell, B.
  • Chang, C. J.
  • Kalyanaraman, B.
  • Rhee, S. G.
  • Thornalley, P. J.
  • Partridge, L.
  • Gems, D.
  • Nystrom, T.
  • Belousov, V.
  • Schumacker, P. T.
  • Winterbourn, C. C.
(2011) Cell Metab, 13(4): 361-6

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

Impaired nigrostriatal function precedes behavioral deficits in a genetic mitochondrial model of Parkinson's disease

  • Good, C. H.
  • Hoffman, A. F.
  • Hoffer, B. J.
  • Chefer, V. I.
  • Shippenberg, T. S.
  • Backman, C. M.
  • Larsson, N. G.
  • Olson, L.
  • Gellhaar, S.
  • Galter, D.
  • Lupica, C. R.
(2011) Faseb j, 25(4): 1333-44

Sequence-specific stalling of DNA polymerase gamma and the effects of mutations causing progressive ophthalmoplegia

  • Atanassova, N.
  • Fuste, J. M.
  • Wanrooij, S.
  • Macao, B.
  • Goffart, S.
  • Backstrom, S.
  • Farge, G.
  • Khvorostov, I.
  • Larsson, N. G.
  • Spelbrink, J. N.
  • Falkenberg, M.
(2011) Hum Mol Genet, 20(6): 1212-23

A common variant in TFB1M is associated with reduced insulin secretion and increased future risk of type 2 diabetes

  • Koeck, T.
  • Olsson, A. H.
  • Nitert, M. D.
  • Sharoyko, V. V.
  • Ladenvall, C.
  • Kotova, O.
  • Reiling, E.
  • Ronn, T.
  • Parikh, H.
  • Taneera, J.
  • Eriksson, J. G.
  • Metodiev, M. D.
  • Larsson, N. G.
  • Balhuizen, A.
  • Luthman, H.
  • Stancakova, A.
  • Kuusisto, J.
  • Laakso, M.
  • Poulsen, P.
  • Vaag, A.
  • Groop, L.
  • Lyssenko, V.
  • Mulder, H.
  • Ling, C.
(2011) Cell Metab, 13(1): 80-91

High brain lactate is a hallmark of aging and caused by a shift in the lactate dehydrogenase A/B ratio

  • Ross, J. M.
  • Oberg, J.
  • Brene, S.
  • Coppotelli, G.
  • Terzioglu, M.
  • Pernold, K.
  • Goiny, M.
  • Sitnikov, R.
  • Kehr, J.
  • Trifunovic, A.
  • Larsson, N. G.
  • Hoffer, B. J.
  • Olson, L.
(2010) Proc Natl Acad Sci U S A, 107(46): 20087-92

Maintenance of respiratory chain function in mouse hearts with severely impaired mtDNA transcription

  • Freyer, C.
  • Park, C. B.
  • Ekstrand, M. I.
  • Shi, Y.
  • Khvorostova, J.
  • Wibom, R.
  • Falkenberg, M.
  • Gustafsson, C. M.
  • Larsson, N. G.
(2010) Nucleic Acids Res, 38(19): 6577-88

LRPPRC is a mitochondrial matrix protein that is conserved in metazoans

  • Sterky, F. H.
  • Ruzzenente, B.
  • Gustafsson, C. M.
  • Samuelsson, T.
  • Larsson, N. G.
(2010) Biochem Biophys Res Commun, 398(4): 759-64

MTERF1 gives mtDNA an unusual twist

  • Gustafsson, C. M.
  • Larsson, N. G.
(2010) Cell Metab, 12(1): 3-4

Somatic mitochondrial DNA mutations in mammalian aging

  • Larsson, N. G.
(2010) Annu Rev Biochem, 79: 683-706

MitoPark mice mirror the slow progression of key symptoms and L-DOPA response in Parkinson's disease

  • Galter, D.
  • Pernold, K.
  • Yoshitake, T.
  • Lindqvist, E.
  • Hoffer, B.
  • Kehr, J.
  • Larsson, N. G.
  • Olson, L.
(2010) Genes Brain Behav, 9(2): 173-81

Two novel mutations in thymidine kinase-2 cause early onset fatal encephalomyopathy and severe mtDNA depletion

  • Lesko, N.
  • Naess, K.
  • Wibom, R.
  • Solaroli, N.
  • Nennesmo, I.
  • von Dobeln, U.
  • Karlsson, A.
  • Larsson, N. G.
(2010) Neuromuscul Disord, 20(3): 198-203

Response: Point Mutations Are Causing Progeroid Phenotypes in the mtDNA Mutator Mouse

  • Edgar, D.
  • Larsson, N. G.
  • Trifunovic, A.
(2010) Cell Metab, 11(1): 93

Point mutations are causing progeroid phenotypes in the mtDNA mutator mouse

  • Edgar, D.
  • Larsson, N. G.
  • Trifunovic, A.
(2010) Cell Metab, 11(1): 1

Nurr1 is required for maintenance of maturing and adult midbrain dopamine neurons

  • Kadkhodaei, B.
  • Ito, T.
  • Joodmardi, E.
  • Mattsson, B.
  • Rouillard, C.
  • Carta, M.
  • Muramatsu, S.
  • Sumi-Ichinose, C.
  • Nomura, T.
  • Metzger, D.
  • Chambon, P.
  • Lindqvist, E.
  • Larsson, N. G.
  • Olson, L.
  • Bjorklund, A.
  • Ichinose, H.
  • Perlmann, T.
(2009) J Neurosci, 29(50): 15923-32

Structure casts light on mtDNA replication

  • Falkenberg, M.
  • Larsson, N. G.
(2009) Cell, 139(2): 231-3

Random point mutations with major effects on protein-coding genes are the driving force behind premature aging in mtDNA mutator mice

  • Edgar, D.
  • Shabalina, I.
  • Camara, Y.
  • Wredenberg, A.
  • Calvaruso, M. A.
  • Nijtmans, L.
  • Nedergaard, J.
  • Cannon, B.
  • Larsson, N. G.
  • Trifunovic, A.
(2009) Cell Metab, 10(2): 131-8

Sensory ataxic neuropathy in golden retriever dogs is caused by a deletion in the mitochondrial tRNATyr gene

  • Baranowska, I.
  • Jaderlund, K. H.
  • Nennesmo, I.
  • Holmqvist, E.
  • Heidrich, N.
  • Larsson, N. G.
  • Andersson, G.
  • Wagner, E. G.
  • Hedhammar, A.
  • Wibom, R.
  • Andersson, L.
(2009) PLoS Genet, 5(5): e1000499

MTERF2 is a nucleoid component in mammalian mitochondria

  • Pellegrini, M.
  • Asin-Cayuela, J.
  • Erdjument-Bromage, H.
  • Tempst, P.
  • Larsson, N. G.
  • Gustafsson, C. M.
(2009) Biochim Biophys Acta, 1787(5): 296-302

Methylation of 12S rRNA is necessary for in vivo stability of the small subunit of the mammalian mitochondrial ribosome

  • Metodiev, M. D.
  • Lesko, N.
  • Park, C. B.
  • Camara, Y.
  • Shi, Y.
  • Wibom, R.
  • Hultenby, K.
  • Gustafsson, C. M.
  • Larsson, N. G.
(2009) Cell Metab, 9(4): 386-97

Introduction: Euromit VII--summarizing 50 years of research on mitochondrial disease

  • Larsson, N. G.
(2009) J Intern Med, 265(2): 170-3

Increased mitochondrial Ca2+ and decreased sarcoplasmic reticulum Ca2+ in mitochondrial myopathy

  • Aydin, J.
  • Andersson, D. C.
  • Hanninen, S. L.
  • Wredenberg, A.
  • Tavi, P.
  • Park, C. B.
  • Larsson, N. G.
  • Bruton, J. D.
  • Westerblad, H.
(2009) Hum Mol Genet, 18(2): 278-88

MtDNA mutations are a common cause of severe disease phenotypes in children with Leigh syndrome

  • Naess, K.
  • Freyer, C.
  • Bruhn, H.
  • Wibom, R.
  • Malm, G.
  • Nennesmo, I.
  • von Dobeln, U.
  • Larsson, N. G.
(2009) Biochim Biophys Acta, 1787(5): 484-90

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

Age-associated mosaic respiratory chain deficiency causes trans-neuronal degeneration

  • Dufour, E.
  • Terzioglu, M.
  • Sterky, F. H.
  • Sorensen, L.
  • Galter, D.
  • Olson, L.
  • Wilbertz, J.
  • Larsson, N. G.
(2008) Hum Mol Genet, 17(10): 1418-26

Complex I: a complex gateway to the powerhouse

  • Sterky, F. H.
  • Larsson, N. G.
(2008) Cell Metab, 7(4): 278-9

Mitochondrial dysfunction as a cause of ageing

  • Trifunovic, A.
  • Larsson, N. G.
(2008) J Intern Med, 263(2): 167-78

Reactive oxygen species and fatigue-induced prolonged low-frequency force depression in skeletal muscle fibres of rats, mice and SOD2 overexpressing mice

  • Bruton, J. D.
  • Place, N.
  • Yamada, T.
  • Silva, J. P.
  • Andrade, F. H.
  • Dahlstedt, A. J.
  • Zhang, S. J.
  • Katz, A.
  • Larsson, N. G.
  • Westerblad, H.
(2008) J Physiol, 586(1): 175-84

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

Mitochondrial dysfunction in mammalian ageing

  • Terzioglu, M.
  • Larsson, N. G.
(2007) Novartis Found Symp, 287: 197-208; discussion 208-13

Is energy deficiency good in moderation?

  • Freyer, C.
  • Larsson, N. G.
(2007) Cell, 131(3): 448-50

Somatic mtDNA mutations cause progressive hearing loss in the mouse

  • Niu, X.
  • Trifunovic, A.
  • Larsson, N. G.
  • Canlon, B.
(2007) Exp Cell Res, 313(18): 3924-34

MTERF3 is a negative regulator of mammalian mtDNA transcription

  • Park, C. B.
  • Asin-Cayuela, J.
  • Camara, Y.
  • Shi, Y.
  • Pellegrini, M.
  • Gaspari, M.
  • Wibom, R.
  • Hultenby, K.
  • Erdjument-Bromage, H.
  • Tempst, P.
  • Falkenberg, M.
  • Gustafsson, C. M.
  • Larsson, N. G.
(2007) Cell, 130(2): 273-85

DNA replication and transcription in mammalian mitochondria

  • Falkenberg, M.
  • Larsson, N. G.
  • Gustafsson, C. M.
(2007) Annu Rev Biochem, 76: 679-99

Progressive parkinsonism in mice with respiratory-chain-deficient dopamine neurons

  • Ekstrand, M. I.
  • Terzioglu, M.
  • Galter, D.
  • Zhu, S.
  • Hofstetter, C.
  • Lindqvist, E.
  • Thams, S.
  • Bergstrand, A.
  • Hansson, F. S.
  • Trifunovic, A.
  • Hoffer, B.
  • Cullheim, S.
  • Mohammed, A. H.
  • Olson, L.
  • Larsson, N. G.
(2007) Proc Natl Acad Sci U S A, 104(4): 1325-30

Proteolytic processing of OPA1 links mitochondrial dysfunction to alterations in mitochondrial morphology

  • Duvezin-Caubet, S.
  • Jagasia, R.
  • Wagener, J.
  • Hofmann, S.
  • Trifunovic, A.
  • Hansson, A.
  • Chomyn, A.
  • Bauer, M. F.
  • Attardi, G.
  • Larsson, N. G.
  • Neupert, W.
  • Reichert, A. S.
(2006) J Biol Chem, 281(49): 37972-9

Respiratory chain dysfunction in skeletal muscle does not cause insulin resistance

  • Wredenberg, A.
  • Freyer, C.
  • Sandstrom, M. E.
  • Katz, A.
  • Wibom, R.
  • Westerblad, H.
  • Larsson, N. G.
(2006) Biochem Biophys Res Commun, 350(1): 202-7

Somatic mtDNA mutations cause aging phenotypes without affecting reactive oxygen species production

  • Trifunovic, A.
  • Hansson, A.
  • Wredenberg, A.
  • Rovio, A. T.
  • Dufour, E.
  • Khvorostov, I.
  • Spelbrink, J. N.
  • Wibom, R.
  • Jacobs, H. T.
  • Larsson, N. G.
(2005) Proc Natl Acad Sci U S A, 102(50): 17993-8

SOD2 overexpression: enhanced mitochondrial tolerance but absence of effect on UCP activity

  • Silva, J. P.
  • Shabalina, I. G.
  • Dufour, E.
  • Petrovic, N.
  • Backlund, E. C.
  • Hultenby, K.
  • Wibom, R.
  • Nedergaard, J.
  • Cannon, B.
  • Larsson, N. G.
(2005) Embo j, 24(23): 4061-70

A family of putative transcription termination factors shared amongst metazoans and plants

  • Linder, T.
  • Park, C. B.
  • Asin-Cayuela, J.
  • Pellegrini, M.
  • Larsson, N. G.
  • Falkenberg, M.
  • Samuelsson, T.
  • Gustafsson, C. M.
(2005) Curr Genet, 48(4): 265-9

Secondary metabolic effects in complex I deficiency

  • Esteitie, N.
  • Hinttala, R.
  • Wibom, R.
  • Nilsson, H.
  • Hance, N.
  • Naess, K.
  • Tear-Fahnehjelm, K.
  • von Dobeln, U.
  • Majamaa, K.
  • Larsson, N. G.
(2005) Ann Neurol, 58(4): 544-52

Abnormal Ca(2+) release and catecholamine-induced arrhythmias in mitochondrial cardiomyopathy

  • Tavi, P.
  • Hansson, A.
  • Zhang, S. J.
  • Larsson, N. G.
  • Westerblad, H.
(2005) Hum Mol Genet, 14(8): 1069-76

The transcription machinery in mammalian mitochondria

  • Gaspari, M.
  • Larsson, N. G.
  • Gustafsson, C. M.
(2004) Biochim Biophys Acta, 1659(2-3): 148-52

The mitochondrial RNA polymerase contributes critically to promoter specificity in mammalian cells

  • Gaspari, M.
  • Falkenberg, M.
  • Larsson, N. G.
  • Gustafsson, C. M.
(2004) Embo j, 23(23): 4606-14

Defective assembly of the respiratory chain

  • Esteitie, N.
  • Larsson, N. G.
(2004) Acta Paediatr, 93(10): 1268-70

Understanding aging: revealing order out of chaos

  • Dufour, E.
  • Larsson, N. G.
(2004) Biochim Biophys Acta, 1658(1-2): 122-32

Premature ageing in mice expressing defective mitochondrial DNA polymerase

  • Trifunovic, A.
  • Wredenberg, A.
  • Falkenberg, M.
  • Spelbrink, J. N.
  • Rovio, A. T.
  • Bruder, C. E.
  • Bohlooly, Y. M.
  • Gidlof, S.
  • Oldfors, A.
  • Wibom, R.
  • Tornell, J.
  • Jacobs, H. T.
  • Larsson, N. G.
(2004) Nature, 429(6990): 417-23

Mitochondrial transcription factor A regulates mtDNA copy number in mammals

  • Ekstrand, M. I.
  • Falkenberg, M.
  • Rantanen, A.
  • Park, C. B.
  • Gaspari, M.
  • Hultenby, K.
  • Rustin, P.
  • Gustafsson, C. M.
  • Larsson, N. G.
(2004) Hum Mol Genet, 13(9): 935-44

A switch in metabolism precedes increased mitochondrial biogenesis in respiratory chain-deficient mouse hearts

  • Hansson, A.
  • Hance, N.
  • Dufour, E.
  • Rantanen, A.
  • Hultenby, K.
  • Clayton, D. A.
  • Wibom, R.
  • Larsson, N. G.
(2004) Proc Natl Acad Sci U S A, 101(9): 3136-41

Characterization of the mouse genes for mitochondrial transcription factors B1 and B2

  • Rantanen, A.
  • Gaspari, M.
  • Falkenberg, M.
  • Gustafsson, C. M.
  • Larsson, N. G.
(2003) Mamm Genome, 14(1): 1-6

Increased mitochondrial mass in mitochondrial myopathy mice

  • Wredenberg, A.
  • Wibom, R.
  • Wilhelmsson, H.
  • Graff, C.
  • Wiener, H. H.
  • Burden, S. J.
  • Oldfors, A.
  • Westerblad, H.
  • Larsson, N. G.
(2002) Proc Natl Acad Sci U S A, 99(23): 15066-71

Leber hereditary optic neuropathy: a nuclear solution of a mitochondrial problem

  • Larsson, N. G.
(2002) Ann Neurol, 52(5): 529-30

Mitochondrial diseases

  • Graff, C.
  • Bui, T. H.
  • Larsson, N. G.
(2002) Best Pract Res Clin Obstet Gynaecol, 16(5): 715-28

Manipulation of mitochondrial DNA gene expression in the mouse

  • Silva, J. P.
  • Larsson, N. G.
(2002) Biochim Biophys Acta, 1555(1-3): 106-10

Mitochondrial transcription factors B1 and B2 activate transcription of human mtDNA

  • Falkenberg, M.
  • Gaspari, M.
  • Rantanen, A.
  • Trifunovic, A.
  • Larsson, N. G.
  • Gustafsson, C. M.
(2002) Nat Genet, 31(3): 289-94

Tissue-specific knockout model for study of mitochondrial DNA mutation disorders

  • Trifunovic, A.
  • Larsson, N. G.
(2002) Methods Enzymol, 353: 409-21

Breeding and genotyping of Tfam conditional knockout mice

  • Ekstrand, M.
  • Larsson, N. G.
(2002) Methods Mol Biol, 197: 391-400

Animal models for respiratory chain disease

  • Larsson, N. G.
  • Rustin, P.
(2001) Trends Mol Med, 7(12): 578-81

Late-onset corticohippocampal neurodepletion attributable to catastrophic failure of oxidative phosphorylation in MILON mice

  • Sorensen, L.
  • Ekstrand, M.
  • Silva, J. P.
  • Lindqvist, E.
  • Xu, B.
  • Rustin, P.
  • Olson, L.
  • Larsson, N. G.
(2001) J Neurosci, 21(20): 8082-90

Downregulation of Tfam and mtDNA copy number during mammalian spermatogenesis

  • Rantanen, A.
  • Jansson, M.
  • Oldfors, A.
  • Larsson, N. G.
(2001) Mamm Genome, 12(10): 787-92

Mitochondrial myopathies

  • Larsson, N. G.
  • Oldfors, A.
(2001) Acta Physiol Scand, 171(3): 385-93

Increased in vivo apoptosis in cells lacking mitochondrial DNA gene expression

  • Wang, J.
  • Silva, J. P.
  • Gustafsson, C. M.
  • Rustin, P.
  • Larsson, N. G.
(2001) Proc Natl Acad Sci U S A, 98(7): 4038-43

Impaired insulin secretion and beta-cell loss in tissue-specific knockout mice with mitochondrial diabetes

  • Silva, J. P.
  • Kohler, M.
  • Graff, C.
  • Oldfors, A.
  • Magnuson, M. A.
  • Berggren, P. O.
  • Larsson, N. G.
(2000) Nat Genet, 26(3): 336-40

Regulation of mitochondrial DNA copy number during spermatogenesis

  • Rantanen, A.
  • Larsson, N. G.
(2000) Hum Reprod, 15 Suppl 2: 86-91

Complex genetic counselling and prenatal analysis in a woman with external ophthalmoplegia and deleted mtDNA

  • Graff, C.
  • Wredenberg, A.
  • Silva, J. P.
  • Bui, T. H.
  • Borg, K.
  • Larsson, N. G.
(2000) Prenat Diagn, 20(5): 426-31

Genetic modification of survival in tissue-specific knockout mice with mitochondrial cardiomyopathy

  • Li, H.
  • Wang, J.
  • Wilhelmsson, H.
  • Hansson, A.
  • Thoren, P.
  • Duffy, J.
  • Rustin, P.
  • Larsson, N. G.
(2000) Proc Natl Acad Sci U S A, 97(7): 3467-72

Revolution in mitochondrial medicine

  • Larsson, N. G.
  • Luft, R.
(1999) FEBS Lett, 455(3): 199-202

Mitochondrial medicine--recent advances

  • Graff, C.
  • Clayton, D. A.
  • Larsson, N. G.
(1999) J Intern Med, 246(1): 11-23

Dilated cardiomyopathy and atrioventricular conduction blocks induced by heart-specific inactivation of mitochondrial DNA gene expression

  • Wang, J.
  • Wilhelmsson, H.
  • Graff, C.
  • Li, H.
  • Oldfors, A.
  • Rustin, P.
  • Bruning, J. C.
  • Kahn, C. R.
  • Clayton, D. A.
  • Barsh, G. S.
  • Thoren, P.
  • Larsson, N. G.
(1999) Nat Genet, 21(1): 133-7

Mitochondrial transcription factor A is necessary for mtDNA maintenance and embryogenesis in mice

  • Larsson, N. G.
  • Wang, J.
  • Wilhelmsson, H.
  • Oldfors, A.
  • Rustin, P.
  • Lewandoski, M.
  • Barsh, G. S.
  • Clayton, D. A.
(1998) Nat Genet, 18(3): 231-6

Structure and chromosomal localization of the mouse mitochondrial transcription factor A gene (Tfam)

  • Larsson, N. G.
  • Barsh, G. S.
  • Clayton, D. A.
(1997) Mamm Genome, 8(2): 139-40

Down-regulation of mitochondrial transcription factor A during spermatogenesis in humans

  • Larsson, N. G.
  • Oldfors, A.
  • Garman, J. D.
  • Barsh, G. S.
  • Clayton, D. A.
(1997) Hum Mol Genet, 6(2): 185-91

A single mouse gene encodes the mitochondrial transcription factor A and a testis-specific nuclear HMG-box protein

  • Larsson, N. G.
  • Garman, J. D.
  • Oldfors, A.
  • Barsh, G. S.
  • Clayton, D. A.
(1996) Nat Genet, 13(3): 296-302

Fatal mitochondrial myopathy, lactic acidosis, and complex I deficiency associated with a heteroplasmic A --> G mutation at position 3251 in the mitochondrial tRNALeu(UUR) gne

  • Houshmand, M.
  • Larsson, N. G.
  • Oldfors, A.
  • Tulinius, M.
  • Holme, E.
(1996) Hum Genet, 97(3): 269-73

De novo mutation in the mitochondrial ATP synthase subunit 6 gene (T8993G) with rapid segregation resulting in Leigh syndrome in the offspring

  • Tulinius, M. H.
  • Houshmand, M.
  • Larsson, N. G.
  • Holme, E.
  • Oldfors, A.
  • Holmberg, E.
  • Wahlstrom, J.
(1995) Hum Genet, 96(3): 290-4

Mitochondrial DNA deletions in muscle fibers in inclusion body myositis

  • Oldfors, A.
  • Moslemi, A. R.
  • Fyhr, I. M.
  • Holme, E.
  • Larsson, N. G.
  • Lindberg, C.
(1995) J Neuropathol Exp Neurol, 54(4): 581-7

Inheritance and expression of mitochondrial DNA point mutations

  • Holme, E.
  • Tulinius, M. H.
  • Larsson, N. G.
  • Oldfors, A.
(1995) Biochim Biophys Acta, 1271(1): 249-52

Use of primary cultures and continuous cell lines to study effects on astrocytic regulatory functions

  • Walum, E.
  • Eriksson, G.
  • Peterson, A.
  • Holme, E.
  • Larsson, N. G.
  • Eriksson, C.
  • el-Shamy, W.
(1995) Clin Exp Pharmacol Physiol, 22(4): 284-7

Atypical presentation of multisystem disorders in two girls with mitochondrial DNA deletions

  • Tulinius, M. H.
  • Oldfors, A.
  • Holme, E.
  • Larsson, N. G.
  • Houshmand, M.
  • Fahleson, P.
  • Sigstrom, L.
  • Kristiansson, B.
(1995) Eur J Pediatr, 154(1): 35-42

Tissue distribution and disease manifestations of the tRNA(Lys) A-->G(8344) mitochondrial DNA mutation in a case of myoclonus epilepsy and ragged red fibres

  • Oldfors, A.
  • Holme, E.
  • Tulinius, M.
  • Larsson, N. G.
(1995) Acta Neuropathol, 90(3): 328-33

Pathogenetic aspects of the A8344G mutation of mitochondrial DNA associated with MERRF syndrome and multiple symmetric lipomas

  • Larsson, N. G.
  • Tulinius, M. H.
  • Holme, E.
  • Oldfors, A.
(1995) Muscle Nerve Suppl, 3: S102-6

Molecular genetic aspects of human mitochondrial disorders

  • Larsson, N. G.
  • Clayton, D. A.
(1995) Annu Rev Genet, 29: 151-78

Low levels of mitochondrial transcription factor A in mitochondrial DNA depletion

  • Larsson, N. G.
  • Oldfors, A.
  • Holme, E.
  • Clayton, D. A.
(1994) Biochem Biophys Res Commun, 200(3): 1374-81

Automatic sequencing of mitochondrial tRNA genes in patients with mitochondrial encephalomyopathy

  • Houshmand, M.
  • Larsson, N. G.
  • Holme, E.
  • Oldfors, A.
  • Tulinius, M. H.
  • Andersen, O.
(1994) Biochim Biophys Acta, 1226(1): 49-55

Mitochondrial DNA deletions in inclusion body myositis

  • Oldfors, A.
  • Larsson, N. G.
  • Lindberg, C.
  • Holme, E.
(1993) Brain, 116 ( Pt 2): 325-36

Multiple symmetric lipomas with high levels of mtDNA with the tRNA(Lys) A-->G(8344) mutation as the only manifestation of disease in a carrier of myoclonus epilepsy and ragged-red fibers (MERRF) syndrome

  • Holme, E.
  • Larsson, N. G.
  • Oldfors, A.
  • Tulinius, M.
  • Sahlin, P.
  • Stenman, G.
(1993) Am J Hum Genet, 52(3): 551-6

Segregation and manifestations of the mtDNA tRNA(Lys) A-->G(8344) mutation of myoclonus epilepsy and ragged-red fibers (MERRF) syndrome

  • Larsson, N. G.
  • Tulinius, M. H.
  • Holme, E.
  • Oldfors, A.
  • Andersen, O.
  • Wahlstrom, J.
  • Aasly, J.
(1992) Am J Hum Genet, 51(6): 1201-12

Mitochondrial ATP-synthase deficiency in a child with 3-methylglutaconic aciduria

  • Holme, E.
  • Greter, J.
  • Jacobson, C. E.
  • Larsson, N. G.
  • Lindstedt, S.
  • Nilsson, K. O.
  • Oldfors, A.
  • Tulinius, M.
(1992) Pediatr Res, 32(6): 731-5

Multiple short direct repeats associated with single mtDNA deletions

  • Larsson, N. G.
  • Holme, E.
(1992) Biochim Biophys Acta, 1139(4): 311-4

Mitochondrial DNA deletions and cytochrome c oxidase deficiency in muscle fibres

  • Oldfors, A.
  • Larsson, N. G.
  • Holme, E.
  • Tulinius, M.
  • Kadenbach, B.
  • Droste, M.
(1992) J Neurol Sci, 110(1-2): 169-77

Lack of transmission of deleted mtDNA from a woman with Kearns-Sayre syndrome to her child

  • Larsson, N. G.
  • Eiken, H. G.
  • Boman, H.
  • Holme, E.
  • Oldfors, A.
  • Tulinius, M. H.
(1992) Am J Hum Genet, 50(2): 360-3

Leber's hereditary optic neuropathy and complex I deficiency in muscle

  • Larsson, N. G.
  • Andersen, O.
  • Holme, E.
  • Oldfors, A.
  • Wahlstrom, J.
(1991) Ann Neurol, 30(5): 701-8

Mitochondrial encephalomyopathies in childhood. I. Biochemical and morphologic investigations

  • Tulinius, M. H.
  • Holme, E.
  • Kristiansson, B.
  • Larsson, N. G.
  • Oldfors, A.
(1991) J Pediatr, 119(2): 242-50

Mitochondrial encephalomyopathies in childhood. II. Clinical manifestations and syndromes

  • Tulinius, M. H.
  • Holme, E.
  • Kristiansson, B.
  • Larsson, N. G.
  • Oldfors, A.
(1991) J Pediatr, 119(2): 251-9

Progressive increase of the mutated mitochondrial DNA fraction in Kearns-Sayre syndrome

  • Larsson, N. G.
  • Holme, E.
  • Kristiansson, B.
  • Oldfors, A.
  • Tulinius, M.
(1990) Pediatr Res, 28(2): 131-6

Neuropathology in Kearns-Sayre syndrome

  • Oldfors, A.
  • Fyhr, I. M.
  • Holme, E.
  • Larsson, N. G.
  • Tulinius, M.
(1990) Acta Neuropathol, 80(5): 541-6

[DNA diagnosis of mitochondrial diseases is now possible]

  • Larsson, N. G.
  • Holme, E.
  • Tulinius, M. H.
(1989) Lakartidningen, 86(48): 4235-6

Stable transfection of a human lymphoma line by sub-genomic fragments of Epstein-Barr virus DNA to measure humoral and cellular immunity to the corresponding proteins

  • Welinder, C.
  • Larsson, N. G.
  • Szigeti, R.
  • Ehlin-Henriksson, B.
  • Henle, G.
  • Henle, W.
  • Klein, G.
  • Ricksten, A.
  • Rymo, L.
  • Sulitzeanu, D.
(1987) Int J Cancer, 40(3): 389-95

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