| LRPPRC is necessary for polyadenylation and coordination of translation of mitochondrial mRNAs B Ruzzenente, MD Metodiev, A Wredenberg, A Bratic, CB Park, Y Cámara, ... The EMBO journal 31 (2), 443-456, 2012 | 368 | 2012 |
| MTERF4 regulates translation by targeting the methyltransferase NSUN4 to the mammalian mitochondrial ribosome Y Cámara, J Asin-Cayuela, CB Park, MD Metodiev, Y Shi, B Ruzzenente, ... Cell metabolism 13 (5), 527-539, 2011 | 307 | 2011 |
| NSUN4 is a dual function mitochondrial protein required for both methylation of 12S rRNA and coordination of mitoribosomal assembly MD Metodiev, H Spåhr, P Loguercio Polosa, C Meharg, C Becker, ... PLoS genetics 10 (2), e1004110, 2014 | 303 | 2014 |
| TWINKLE is an essential mitochondrial helicase required for synthesis of nascent D-loop strands and complete mtDNA replication D Milenkovic, S Matic, I Kühl, B Ruzzenente, C Freyer, E Jemt, CB Park, ... Human molecular genetics 22 (10), 1983-1993, 2013 | 183 | 2013 |
| MTERF1 binds mtDNA to prevent transcriptional interference at the light-strand promoter but is dispensable for rRNA gene transcription regulation M Terzioglu, B Ruzzenente, J Harmel, A Mourier, E Jemt, MD Lopez, ... Cell metabolism 17 (4), 618-626, 2013 | 168 | 2013 |
| Mitochondrial fusion is required for regulation of mitochondrial DNA replication E Silva Ramos, E Motori, C Brüser, I Kühl, A Yeroslaviz, B Ruzzenente, ... PLoS genetics 15 (6), e1008085, 2019 | 164 | 2019 |
| CLPP coordinates mitoribosomal assembly through the regulation of ERAL 1 levels K Szczepanowska, P Maiti, A Kukat, E Hofsetz, H Nolte, K Senft, C Becker, ... The EMBO journal 35 (23), 2566-2583, 2016 | 152 | 2016 |
| Loss of LRPPRC causes ATP synthase deficiency A Mourier, B Ruzzenente, T Brandt, W Kühlbrandt, NG Larsson Human molecular genetics 23 (10), 2580-2592, 2014 | 136 | 2014 |
| Biallelic mutations in MRPS34 lead to instability of the small mitoribosomal subunit and Leigh syndrome NJ Lake, BD Webb, DA Stroud, TR Richman, B Ruzzenente, AG Compton, ... The American Journal of Human Genetics 101 (2), 239-254, 2017 | 132 | 2017 |
| The respiratory chain supercomplex organization is independent of COX7a2l isoforms A Mourier, S Matic, B Ruzzenente, NG Larsson, D Milenkovic Cell metabolism 20 (6), 1069-1075, 2014 | 131 | 2014 |
| MTERF3 regulates mitochondrial ribosome biogenesis in invertebrates and mammals A Wredenberg, M Lagouge, A Bratic, MD Metodiev, H Spåhr, A Mourier, ... PLoS genetics 9 (1), e1003178, 2013 | 126 | 2013 |
| LRPPRC-mediated folding of the mitochondrial transcriptome SJ Siira, H Spåhr, AMJ Shearwood, B Ruzzenente, NG Larsson, ... Nature communications 8 (1), 1532, 2017 | 125 | 2017 |
| Bi-allelic mutations in the mitochondrial ribosomal protein MRPS2 cause sensorineural hearing loss, hypoglycemia, and multiple OXPHOS complex deficiencies T Gardeitchik, M Mohamed, B Ruzzenente, D Karall, S Guerrero-Castillo, ... The American Journal of Human Genetics 102 (4), 685-695, 2018 | 80 | 2018 |
| The Bicoid Stability Factor Controls Polyadenylation and Expression of Specific Mitochondrial mRNAs in Drosophila melanogaster A Bratic, A Wredenberg, S Grönke, JB Stewart, A Mourier, B Ruzzenente, ... PLoS genetics 7 (10), e1002324, 2011 | 71 | 2011 |
| LRPPRC is a mitochondrial matrix protein that is conserved in metazoans FH Sterky, B Ruzzenente, CM Gustafsson, T Samuelsson, NG Larsson Biochemical and biophysical research communications 398 (4), 759-764, 2010 | 69 | 2010 |
| Mutations in complex I assembly factor TMEM126B result in muscle weakness and isolated complex I deficiency L Sánchez-Caballero, B Ruzzenente, L Bianchi, Z Assouline, G Barcia, ... The American Journal of Human Genetics 99 (1), 208-216, 2016 | 58 | 2016 |
| POLRMT does not transcribe nuclear genes I Kühl, C Kukat, B Ruzzenente, D Milenkovic, A Mourier, M Miranda, ... Nature 514 (7521), E7-E11, 2014 | 44 | 2014 |
| The leucine-rich pentatricopeptide repeat-containing protein (LRPPRC) does not activate transcription in mammalian mitochondria J Harmel, B Ruzzenente, M Terzioglu, H Spåhr, M Falkenberg, ... Journal of Biological Chemistry 288 (22), 15510-15519, 2013 | 44 | 2013 |
| Mouse models for mitochondrial diseases B Ruzzenente, A Rötig, MD Metodiev Human molecular genetics 25 (R2), R115-R122, 2016 | 38 | 2016 |
| Mutations in the MRPS28 gene encoding the small mitoribosomal subunit protein bS1m in a patient with intrauterine growth retardation, craniofacial dysmorphism … J Pulman, B Ruzzenente, L Bianchi, M Rio, N Boddaert, A Munnich, ... Human Molecular Genetics 28 (9), 1445-1462, 2019 | 35 | 2019 |
