Publications of H. Spahr

Mehmedovic, M.; Martucci, M.; Spahr, H.; Ishak, L.; Mishra, A.; Sanchez-Sandoval, M. E.; Pardo-Hernandez, C.; Peter, B.; van den Wildenberg, S. M.; Falkenberg, M. et al.; Farge, G.: Disease causing mutation (P178L) in mitochondrial transcription factor A results in impaired mitochondrial transcription initiation. Biochim Biophys Acta Mol Basis Dis, p. 166467 (2022)

Nicholls, T. J.; Spahr, H.; Jiang, S.; Siira, S. J.; Koolmeister, C.; Sharma, S.; Kauppila, J. H. K.; Jiang, M.; Kaever, V.; Rackham, O. et al.; Chabes, A.; Falkenberg, M.; Filipovska, A.; Larsson, N.G.; Gustafsson, C. M.: Dinucleotide Degradation by REXO2 Maintains Promoter Specificity in Mammalian Mitochondria. Mol Cell 76 (5), pp. 784 - 796 e6 (2019)

Gopalakrishna, S.; Pearce, S. F.; Dinan, A. M.; Schober, F. A.; Cipullo, M.; Spahr, H.; Khawaja, A.; Maffezzini, C.; Freyer, C.; Wredenberg, A. et al.; Atanassov, I.; Firth, A. E.; Rorbach, J.: C6orf203 is an RNA-binding protein involved in mitochondrial protein synthesis. Nucleic Acids Res 47 (17), pp. 9386 - 9399 (2019)

Spahr, H.; Chia, T.; Lingford, J. P.; Siira, S. J.; Cohen, S. B.; Filipovska, A.; Rackham, O.: Modular ssDNA binding and inhibition of telomerase activity by designer PPR proteins. Nat Commun 9 (1), p. 2212 (2018)

Perks, K. L.; Rossetti, G.; Kuznetsova, I.; Hughes, L. A.; Ermer, J. A.; Ferreira, N.; Busch, J.; Rudler, D. L.; Spahr, H.; Schöndorf, T. et al.; Shearwood, A. J.; Viola, H. M.; Siira, S. J.; Hool, L. C.; Milenkovic, D.; Larsson, N.G.; Rackham, O.; Filipovska, A.: PTCD1 Is Required for 16S rRNA Maturation Complex Stability and Mitochondrial Ribosome Assembly. Cell Rep 23 (1), pp. 127 - 142 (2018)

Siira, S. J.; Spahr, H.; Shearwood, A. J.; Ruzzenente, B.; Larsson, N.G.; Rackham, O.; Filipovska, A.: LRPPRC-mediated folding of the mitochondrial transcriptome. Nat Commun 8 (1), p. 1532 (2017)

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. et al.; Rackham, O.; Filipovska, A.: Loss of the RNA-binding protein TACO1 causes late-onset mitochondrial dysfunction in mice. Nat Commun 7, p. 11884 (2016)

Kukat, C.; Davies, K. M.; Wurm, C. A.; Spahr, H.; Bonekamp, N. A.; Kühl, I.; Joos, F.; Polosa, P. L.; Park, C. B.; Posse, V. et al.; Falkenberg, M.; Jakobs, S.; Kuhlbrandt, W.; Larsson, N.G.: Cross-strand binding of TFAM to a single mtDNA molecule forms the mitochondrial nucleoid. Proc Natl Acad Sci U S A 112 (36), pp. 11288 - 93 (2015)

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)

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. et al.; Hewitt, L.; Basle, A.; Cross, H. E.; Tong, L.; Lebel, R. R.; Crosby, A. H.; Chrzanowska-Lightowlers, Z. M.; Lightowlers, R. N.: A human mitochondrial poly(A) polymerase mutation reveals the complexities of post-transcriptional mitochondrial gene expression. Hum Mol Genet 23 (23), pp. 6345 - 55 (2014)

Metodiev , M. D.; Spahr, H.; Loguercio Polosa, P.; Meharg, C.; Becker, C.; Altmueller, J.; Habermann, B.H.; Larsson, N.G.; Ruzzenente, B.: NSUN4 is a dual function mitochondrial protein required for both methylation of 12S rRNA and coordination of mitoribosomal assembly. PLoS Genet 10 (2), p. e1004110 (2014)

Metodiev, M.D.; Spahr, H.; Polosa, P. L.; Meharg, C.; Becker, C.; Altmueller, J.; Habermann, B.H.; Larsson, N.G.; Ruzzenente, B.: NSUN4 Is a Dual Function Mitochondrial Protein Required for Both Methylation of 12S rRNA and Coordination of Mitoribosomal Assembly. PLOS GENETICS 10 (2), e1004110 (2014)

Uhler, J. P.; Spahr, H.; Farge, G.; Clavel, S.; Larsson, N.G.; Falkenberg, M.; Samuelsson, T.; Gustafsson, C. M.: The UbL protein UBTD1 stably interacts with the UBE2D family of E2 ubiquitin conjugating enzymes. Biochem Biophys Res Commun 443 (1), pp. 7 - 12 (2013)

Harmel, J.; Ruzzenente, B.; Terzioglu, M.; Spahr, H.; Falkenberg, M.; Larsson, N.G.: The leucine-rich pentatricopeptide repeat-containing protein (LRPPRC) does not activate transcription in mammalian mitochondria. J Biol Chem 288 (22), pp. 15510 - 9 (2013)

Wredenberg, A.; Lagouge, M.; Bratic, A.; Metodiev, M. D.; Spahr, H.; Mourier, A.; Freyer, C.; Ruzzenente, B.; Tain, L. S.; Grönke, S. et al.; Baggio, F.; Kukat, C.; Kremmer, E.; Wibom, R.; Polosa, P. L.; Habermann, B.H.; Partridge, L.; Park, C. B.; Larsson, N.G.: MTERF3 regulates mitochondrial ribosome biogenesis in invertebrates and mammals. PLoS Genet 9 (1), p. e1003178 (2013)

Spahr, H.; Habermann, B.; Gustafsson, C. M.; Larsson, N.G.; Hallberg, B. M.: Structure of the human MTERF4-NSUN4 protein complex that regulates mitochondrial ribosome biogenesis. Proc Natl Acad Sci U S A 109 (38), pp. 15253 - 8 (2012)

Kukat, C.; Wurm, C. A.; Spahr, H.; Falkenberg, M.; Larsson, N.G.; Jakobs, S.: Super-resolution microscopy reveals that mammalian mitochondrial nucleoids have a uniform size and frequently contain a single copy of mtDNA. Proc Natl Acad Sci U S A 108 (33), pp. 13534 - 9 (2011)