Benedetta Ruzzenente
Benedetta Ruzzenente
INSERM, Institut Imagine
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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
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
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
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
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
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
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
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
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
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
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
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
LRPPRC-mediated folding of the mitochondrial transcriptome
SJ Siira, H Spåhr, AMJ Shearwood, B Ruzzenente, NG Larsson, ...
Nature communications 8 (1), 1-11, 2017
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
POLRMT does not transcribe nuclear genes
I Kühl, C Kukat, B Ruzzenente, D Milenkovic, A Mourier, M Miranda, ...
Nature 514 (7521), E7-E11, 2014
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
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
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
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
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
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