Publications of A. Mourier

Kauppila, J. H. K.; Bonekamp, N.; Mourier, A.; Isokallio, M.; Just, A.; Kauppila, T.; Stewart, J.; Larsson, N.G.: Base-excision repair deficiency alone or combined with increased oxidative stress does not increase mtDNA point mutations in mice. Nucleic Acids Res 46 (13), pp. 6642 - 6669 (2018)

Simard, M.-L.; Mourier, A.; Greaves, L. C.; Taylor, R. W.; Stewart, J.: A novel histochemistry assay to assess and quantify focal cytochrome c oxidase deficiency. J Pathol 245 (3), pp. 311 - 323 (2018)

Kühl, I.; Miranda, M.; Atanassov, I.; Kuznetsova, I.; Hinze, Y.; Mourier, A.; Filipovska, A.; Larsson, N.G.: Transcriptomic and proteomic landscape of mitochondrial dysfunction reveals secondary coenzyme Q deficiency in mammals. Elife 6 (2017)

Brandt, T.; Mourier, A.; Tain, L. S.; Partridge, L.; Larsson, N.G.; Kuhlbrandt, W.: Changes of mitochondrial ultrastructure and function during ageing in mice and Drosophila. Elife 6 (2017)

Kauppila, J. H. K.; Baines, H. L.; Bratic, A.; Simard, M.-L.; Freyer, C.; Mourier, A.; Stamp, C.; Filograna, R.; Larsson, N.G.; Greaves, L. C. et al.; Stewart, J. B.: A Phenotype-Driven Approach to Generate Mouse Models with Pathogenic mtDNA Mutations Causing Mitochondrial Disease. Cell Rep 16 (11), pp. 2980 - 90 (2016)

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. et al.; Arenas, J.; Martin, M. A.; Larsson, N.G.; Ugalde, C.: COX7A2L Is a Mitochondrial Complex III Binding Protein that Stabilizes the III2+IV Supercomplex without Affecting Respirasome Formation. Cell Rep 16 (9), pp. 2387 - 98 (2016)

Kühl, I.; Miranda, M.; Posse, V.; Milenkovic, D.; Mourier, A.; Siira, S. J.; Bonekamp, N.; Neumann, U.; Filipovska, A.; Polosa, P. L. et al.; Gustafsson, C. M.; Larsson, N.G.: POLRMT regulates the switch between replication primer formation and gene expression of mammalian mtDNA. Sci Adv 2 (8), p. e1600963 (2016)

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. et al.; Mourier, A.; Milenkovic, D.; Larsson, N.G.; Filipovska, A.: Hierarchical RNA Processing Is Required for Mitochondrial Ribosome Assembly. Cell Rep 16 (7), pp. 1874 - 90 (2016)

Ramos, E.S.; Larsson, N.G.; Mourier, A.: Bioenergetic roles of mitochondrial fusion. Biochim Biophys Acta 1857 (8), pp. 1277 - 83 (2016)

Kauppila, J. H. K.; Baines, H. L.; Bratic, A.; Simard, M.-L.; Freyer, C.; Mourier, A.; Stamp, C.; Filograna, R.; Larsson, N.G.; Greaves, L. C. et al.; Stewart, J. B.: A Phenotype-Driven Approach to Generate Mouse Models with Pathogenic mtDNA Mutations Causing Mitochondrial Disease. Cell reports 16 (11), pp. 2980 - 2990 (2016)

Kühl, I.; Miranda, M.; Posse, V.; Milenkovic, D.; Mourier, A.; Siira, S. J.; Bonekamp, N. A.; Neumann, U.; Filipovska, A.; Polosa, P. L. et al.; Gustafsson, C. M.; Larsson, N.G.: POLRMT regulates the switch between replication primer formation and gene expression of mammalian mtDNA. Science advances 2 (8), p. e1600963 (2016)

Rackham, O.; Busch, J. D.; Matic, S.; Siira, S. .; Kuznetsova, I.; Atanassov, I.; Ermer, J. .; Shearwood, A.-M. J.; Richman, T. .; Stewart, J. B. et al.; Mourier, A.; Milenkovic, D.; Larsson, N.G.; Filipovska, A.: Hierarchical RNA Processing Is Required for Mitochondrial Ribosome Assembly. Cell Reports 16 (7), pp. 1874 - 1890 (2016)

Lagouge, M.; Mourier, A.; Lee, H.J.; Spahr, H.; Wai , T.; Kukat, C.; Ramos, E.S.; Motori, E.; Busch, J. D.; Siira, S. et al.; Kremmer, E.; Filipovska, A.; Larsson, N.G.: SLIRP Regulates the Rate of Mitochondrial Protein Synthesis and Protects LRPPRC from Degradation. PLoS Genet 11 (8), p. e1005423 (2015)

Freyer, C.; Stranneheim, H.; Naess, K.; Mourier, A.; Felser, A.; Maffezzini, C.; Lesko, N.; Bruhn, H.; Engvall, M.; Wibom, R. et al.; Barbaro, M.; Hinze, Y.; Magnusson, M.; Andeer, R.; Zetterstrom, R. H.; von Dobeln, U.; Wredenberg, A.; Wedell, A.: Rescue of primary ubiquinone deficiency due to a novel COQ7 defect using 2,4-dihydroxybensoic acid. J Med Genet 52 (11), pp. 779 - 83 (2015)

Mourier, A.; Motori, E.; Brandt, T.; Lagouge, M.; Atanassov, I.; Galinier, A.; Rappl, G.; Brodesser , S.; Hultenby, K.; Dieterich, C. et al.; Larsson, N.G.: Mitofusin 2 is required to maintain mitochondrial coenzyme Q levels. J Cell Biol 208 (4), pp. 429 - 42 (2015)

Mourier, A.; Matic, S.; Ruzzenente, B.; Larsson, N.G.; Milenkovic, D.: The respiratory chain supercomplex organization is independent of COX7a2l isoforms. Cell Metab 20 (6), pp. 1069 - 75 (2014)

Baggio, F.; Bratic, A.; Mourier, A.; Kauppila, T. E. S.; Tain, L. S.; Kukat, C.; Habermann, B.H.; Partridge, L.; Larsson, N.G.: Drosophila melanogaster LRPPRC2 is involved in coordination of mitochondrial translation. Nucleic Acids Res 42 (22), pp. 13920 - 38 (2014)

Kühl, I.; Kukat, C.; Ruzzenente, B.; Milenkovic, D.; Mourier, A.; Miranda, M.; Koolmeister, C.; Falkenberg, M.; Larsson, N.G.: POLRMT does not transcribe nuclear genes. Nature 514 (7521), pp. E7 - 11 (2014)

Vernochet, C.; Damilano, F.; Mourier, A.; Bezy, O.; Mori, M. A.; Smyth, G.; Rosenzweig, A.; Larsson, N.G.; Kahn, C. R.: Adipose tissue mitochondrial dysfunction triggers a lipodystrophic syndrome with insulin resistance, hepatosteatosis, and cardiovascular complications. Faseb j 28 (10), pp. 4408 - 19 (2014)

Mourier, A.; Ruzzenente, B.; Brandt, T.; Kuhlbrandt, W.; Larsson, N.G.: Loss of LRPPRC causes ATP synthase deficiency. Hum Mol Genet 23 (10), pp. 2580 - 92 (2014)