Publications Department Larsson

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

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

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

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

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

Somatic mitochondrial DNA mutations in mammalian aging

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

MTERF1 gives mtDNA an unusual twist

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

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

Point mutations are causing progeroid phenotypes in the mtDNA mutator mouse

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

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

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

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

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

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

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

Mitochondrial dysfunction as a cause of ageing

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

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

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

Complex I: a complex gateway to the powerhouse

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

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

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

Mitochondrial dysfunction in mammalian ageing

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

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

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

Is energy deficiency good in moderation?

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

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

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

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

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

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

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

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

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

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

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

Understanding aging: revealing order out of chaos

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

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

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

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

Manipulation of mitochondrial DNA gene expression in the mouse

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

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

Breeding and genotyping of Tfam conditional knockout mice

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

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

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

Animal models for respiratory chain disease

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

Mitochondrial myopathies

  • Larsson, N. G.
  • Oldfors, A.
(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.
  • 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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

Multiple short direct repeats associated with single mtDNA deletions

  • Larsson, N. G.
  • Holme, E.
(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.
  • Eiken, H. G.
  • Boman, H.
  • 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