Publications Department Larsson

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

Bioenergetic roles of mitochondrial fusion

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

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

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

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

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

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

Maintenance and Expression of Mammalian Mitochondrial DNA

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

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

Mic10 Oligomerization Pinches off Mitochondrial Cristae

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

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

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

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

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

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

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

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

Keeping mtDNA in shape between generations

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

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

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

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 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

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

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

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

Making proteins in the powerhouse

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

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

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

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

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

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

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

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

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

mtDNA makes a U-turn for the mitochondrial nucleoid

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

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

The role of mitochondria in aging

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

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

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

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

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

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

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

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

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

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

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

Mitochondrial DNA mutations in disease and aging

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

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

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

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

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

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

TFAM forces mtDNA to make a U-turn

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

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

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

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

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

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

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

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(2011) PLoS Genet, 7, 3, e1002028

LRPPRC is a mitochondrial matrix protein that is conserved in metazoans

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(2010) Biochem Biophys Res Commun, 398, 4, 759-64

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

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(2010) Proc Natl Acad Sci U S A, 107, 46, 20087-92

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

  • Lesko, N.
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(2010) Neuromuscul Disord, 20, 3, 198-203

Somatic mitochondrial DNA mutations in mammalian aging

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(2010) Annu Rev Biochem, 79, 683-706

MTERF1 gives mtDNA an unusual twist

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

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

  • Galter, D.
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(2010) Genes Brain Behav, 9, 2, 173-81

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

  • Freyer, C.
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(2010) Nucleic Acids Res, 38, 19, 6577-88

Point mutations are causing progeroid phenotypes in the mtDNA mutator mouse

  • Edgar, D.
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(2010) Cell Metab, 11, 1, 1

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

  • Edgar, D.
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(2010) Cell Metab, 11, 1, 93

MTERF2 is a nucleoid component in mammalian mitochondria

  • Pellegrini, M.
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  • Larsson, N. G.
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(2009) Biochim Biophys Acta, 1787, 5, 296-302

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

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(2009) Biochim Biophys Acta, 1787, 5, 484-90

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

  • Metodiev, M. D.
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(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

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

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(2009) J Neurosci, 29, 50, 15923-32

Structure casts light on mtDNA replication

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(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

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(2009) Cell Metab, 10, 2, 131-8

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

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(2009) PLoS Genet, 5, 5, e1000499

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

  • Aydin, J.
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(2009) Hum Mol Genet, 18, 2, 278-88

Mitochondrial dysfunction as a cause of ageing

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(2008) J Intern Med, 263, 2, 167-78

Strong purifying selection in transmission of mammalian mitochondrial DNA

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(2008) PLoS Biol, 6, 1, e10

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

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(2008) Nat Rev Genet, 9, 9, 657-62

Complex I: a complex gateway to the powerhouse

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(2008) Cell Metab, 7, 4, 278-9

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

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(2008) Hum Mol Genet, 17, 10, 1418-26

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

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(2008) J Physiol, 586, 1, 175-84

Mitochondrial dysfunction in mammalian ageing

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(2007) Novartis Found Symp, 287, 197-208; discussion 208-13

MTERF3 is a negative regulator of mammalian mtDNA transcription

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(2007) Cell, 130, 2, 273-85

Somatic mtDNA mutations cause progressive hearing loss in the mouse

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(2007) Exp Cell Res, 313, 18, 3924-34

Is energy deficiency good in moderation?

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(2007) Cell, 131, 3, 448-50

DNA replication and transcription in mammalian mitochondria

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(2007) Annu Rev Biochem, 76, 679-99

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

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(2007) Proc Natl Acad Sci U S A, 104, 4, 1325-30

Respiratory chain dysfunction in skeletal muscle does not cause insulin resistance

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(2006) Biochem Biophys Res Commun, 350, 1, 202-7

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

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(2006) J Biol Chem, 281, 49, 37972-9

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

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(2005) Proc Natl Acad Sci U S A, 102, 50, 17993-8

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

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(2005) Hum Mol Genet, 14, 8, 1069-76

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

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(2005) Embo j, 24, 23, 4061-70

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

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(2005) Curr Genet, 48, 4, 265-9

Secondary metabolic effects in complex I deficiency

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(2005) Ann Neurol, 58, 4, 544-52

Premature ageing in mice expressing defective mitochondrial DNA polymerase

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(2004) Nature, 429, 6990, 417-23

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

  • Hansson, A.
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(2004) Proc Natl Acad Sci U S A, 101, 9, 3136-41

The transcription machinery in mammalian mitochondria

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(2004) Biochim Biophys Acta, 1659, 2-3, 148-52

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

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(2004) Embo j, 23, 23, 4606-14

Defective assembly of the respiratory chain

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

Mitochondrial transcription factor A regulates mtDNA copy number in mammals

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(2004) Hum Mol Genet, 13, 9, 935-44

Understanding aging: revealing order out of chaos

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(2004) Biochim Biophys Acta, 1658, 1-2, 122-32

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

  • Rantanen, A.
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(2003) Mamm Genome, 14, 1, 1-6

Increased mitochondrial mass in mitochondrial myopathy mice

  • Wredenberg, A.
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(2002) Proc Natl Acad Sci U S A, 99, 23, 15066-71

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

  • Trifunovic, A.
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(2002) Methods Enzymol, 353, 409-21

Manipulation of mitochondrial DNA gene expression in the mouse

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

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.
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(2002) Best Pract Res Clin Obstet Gynaecol, 16, 5, 715-28

Mitochondrial transcription factors B1 and B2 activate transcription of human mtDNA

  • Falkenberg, M.
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(2002) Nat Genet, 31, 3, 289-94

Breeding and genotyping of Tfam conditional knockout mice

  • Ekstrand, M.
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(2002) Methods Mol Biol, 197, 391-400

Increased in vivo apoptosis in cells lacking mitochondrial DNA gene expression

  • Wang, J.
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(2001) Proc Natl Acad Sci U S A, 98, 7, 4038-43

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

  • Sorensen, L.
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(2001) J Neurosci, 21, 20, 8082-90

Downregulation of Tfam and mtDNA copy number during mammalian spermatogenesis

  • Rantanen, A.
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(2001) Mamm Genome, 12, 10, 787-92

Animal models for respiratory chain disease

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

Mitochondrial myopathies

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

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

  • Silva, J. P.
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(2000) Nat Genet, 26, 3, 336-40

Regulation of mitochondrial DNA copy number during spermatogenesis

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

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

  • Li, H.
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(2000) Proc Natl Acad Sci U S A, 97, 7, 3467-72

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

  • Graff, C.
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(2000) Prenat Diagn, 20, 5, 426-31

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

  • Wang, J.
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(1999) Nat Genet, 21, 1, 133-7

Revolution in mitochondrial medicine

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(1999) FEBS Lett, 455, 3, 199-202

Mitochondrial medicine--recent advances

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(1999) J Intern Med, 246, 1, 11-23

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

  • Larsson, N. G.
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(1998) Nat Genet, 18, 3, 231-6

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

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(1997) Hum Mol Genet, 6, 2, 185-91

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

  • Larsson, N. G.
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(1997) Mamm Genome, 8, 2, 139-40

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

  • Larsson, N. G.
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(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

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  • Oldfors, A.
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(1996) Hum Genet, 97, 3, 269-73

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

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  • Eriksson, C.
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(1995) Clin Exp Pharmacol Physiol, 22, 4, 284-7

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

  • Tulinius, M. H.
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(1995) Eur J Pediatr, 154, 1, 35-42

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.
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(1995) Hum Genet, 96, 3, 290-4

Mitochondrial DNA deletions in muscle fibers in inclusion body myositis

  • Oldfors, A.
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(1995) J Neuropathol Exp Neurol, 54, 4, 581-7

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.
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(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.
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(1995) Muscle Nerve Suppl, 3, S102-6

Molecular genetic aspects of human mitochondrial disorders

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(1995) Annu Rev Genet, 29, 151-78

Inheritance and expression of mitochondrial DNA point mutations

  • Holme, E.
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(1995) Biochim Biophys Acta, 1271, 1, 249-52

Low levels of mitochondrial transcription factor A in mitochondrial DNA depletion

  • Larsson, N. G.
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(1994) Biochem Biophys Res Commun, 200, 3, 1374-81

Automatic sequencing of mitochondrial tRNA genes in patients with mitochondrial encephalomyopathy

  • Houshmand, M.
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(1994) Biochim Biophys Acta, 1226, 1, 49-55

Mitochondrial DNA deletions in inclusion body myositis

  • Oldfors, A.
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(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.
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(1993) Am J Hum Genet, 52, 3, 551-6

Mitochondrial DNA deletions and cytochrome c oxidase deficiency in muscle fibres

  • Oldfors, A.
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(1992) J Neurol Sci, 110, 1-2, 169-77

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

  • Larsson, N. G.
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(1992) Am J Hum Genet, 51, 6, 1201-12

Multiple short direct repeats associated with single mtDNA deletions

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

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

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

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

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

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

Neuropathology in Kearns-Sayre syndrome

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

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

[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

*equal contribution; **corresponding author