Benedetta Ruzzenente
Benedetta Ruzzenente
Université Paris Descartes, Institut Imagine
Verified email at inserm.fr
Title
Cited by
Cited by
Year
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
2172012
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
1902011
NSUN4 is a dual function mitochondrial protein required for both methylation of 12S rRNA and coordination of mitoribosomal assembly
MD Metodiev, H Spåhr, PL Polosa, C Meharg, C Becker, J Altmueller, ...
PLoS Genet 10 (2), e1004110, 2014
1332014
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
1062013
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
912014
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 López, ...
Cell metabolism 17 (4), 618-626, 2013
912013
MTERF3 regulates mitochondrial ribosome biogenesis in invertebrates and mammals
A Wredenberg, M Lagouge, A Bratic, MD Metodiev, H Spåhr, A Mourier, ...
PLoS Genet 9 (1), e1003178, 2013
842013
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
672014
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
562010
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
522016
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 Genet 7 (10), e1002324, 2011
522011
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
452017
LRPPRC-mediated folding of the mitochondrial transcriptome
SJ Siira, H Spåhr, AMJ Shearwood, B Ruzzenente, NG Larsson, ...
Nature communications 8 (1), 1-11, 2017
362017
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
322013
POLRMT does not transcribe nuclear genes
I Kühl, C Kukat, B Ruzzenente, D Milenkovic, A Mourier, M Miranda, ...
Nature 514 (7521), E7-E11, 2014
252014
Crystal structures of human and murine deoxyribonucleotidases: insights into recognition of substrates and nucleotide analogues
K Walldén, A Rinaldo-Matthis, B Ruzzenente, C Rampazzo, V Bianchi, ...
Biochemistry 46 (48), 13809-13818, 2007
212007
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
202016
Structural basis for substrate specificity of the human mitochondrial deoxyribonucleotidase
K Walldén, B Ruzzenente, A Rinaldo-Matthis, V Bianchi, P Nordlund
Structure 13 (7), 1081-1088, 2005
202005
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
152018
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
142019
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