Cecilia Mancini, Eriola Hoxha, Luisa Iommarini, Alessandro Brussino, Uwe Richter, Francesca Montarolo, Claudia Cagnoli, Roberta Parolisi, Diana Iulia Gondor Morosini, Valentina Nicolò, Francesca Maltecca, Luisa Muratori, Giulia Ronchi, Stefano Geuna, Francesca Arnaboldi, Elena Donetti, Elisa Giorgio, Simona Cavalieri, Eleonora Di Gregorio, Elisa Pozzi, Marta Ferrero, Evelise Riberi, Giorgio Casari, Fiorella Altruda, Emilia Turco, Giuseppe Gasparre, Brendan J Battersby, Anna Maria Porcelli, Enza Ferrero, Alfredo Brusco, Filippo Tempia. Neurobiol Dis 2019
Times Cited: 15
Times Cited: 15
Times Cited
Times Co-cited
Similarity
Whole-exome sequencing identifies homozygous AFG3L2 mutations in a spastic ataxia-neuropathy syndrome linked to mitochondrial m-AAA proteases.
Tyler Mark Pierson, David Adams, Florian Bonn, Paola Martinelli, Praveen F Cherukuri, Jamie K Teer, Nancy F Hansen, Pedro Cruz, James C Mullikin For The Nisc Comparative Sequencing Program, Robert W Blakesley,[...]. PLoS Genet 2011
Tyler Mark Pierson, David Adams, Florian Bonn, Paola Martinelli, Praveen F Cherukuri, Jamie K Teer, Nancy F Hansen, Pedro Cruz, James C Mullikin For The Nisc Comparative Sequencing Program, Robert W Blakesley,[...]. PLoS Genet 2011
33
The m-AAA protease defective in hereditary spastic paraplegia controls ribosome assembly in mitochondria.
Mark Nolden, Sarah Ehses, Mirko Koppen, Andrea Bernacchia, Elena I Rugarli, Thomas Langer. Cell 2005
Mark Nolden, Sarah Ehses, Mirko Koppen, Andrea Bernacchia, Elena I Rugarli, Thomas Langer. Cell 2005
33
The i-AAA protease YME1L and OMA1 cleave OPA1 to balance mitochondrial fusion and fission.
Ruchika Anand, Timothy Wai, Michael J Baker, Nikolay Kladt, Astrid C Schauss, Elena Rugarli, Thomas Langer. J Cell Biol 2014
Ruchika Anand, Timothy Wai, Michael J Baker, Nikolay Kladt, Astrid C Schauss, Elena Rugarli, Thomas Langer. J Cell Biol 2014
33
Mutations in the mitochondrial protease gene AFG3L2 cause dominant hereditary ataxia SCA28.
Daniela Di Bella, Federico Lazzaro, Alfredo Brusco, Massimo Plumari, Giorgio Battaglia, Annalisa Pastore, Adele Finardi, Claudia Cagnoli, Filippo Tempia, Marina Frontali,[...]. Nat Genet 2010
Daniela Di Bella, Federico Lazzaro, Alfredo Brusco, Massimo Plumari, Giorgio Battaglia, Annalisa Pastore, Adele Finardi, Claudia Cagnoli, Filippo Tempia, Marina Frontali,[...]. Nat Genet 2010
33
Quality control of mitochondrial protein synthesis is required for membrane integrity and cell fitness.
Uwe Richter, Taina Lahtinen, Paula Marttinen, Fumi Suomi, Brendan J Battersby. J Cell Biol 2015
Uwe Richter, Taina Lahtinen, Paula Marttinen, Fumi Suomi, Brendan J Battersby. J Cell Biol 2015
26
AFG3L2 supports mitochondrial protein synthesis and Purkinje cell survival.
Eva R Almajan, Ricarda Richter, Lars Paeger, Paola Martinelli, Esther Barth, Thorsten Decker, Nils-Göran Larsson, Peter Kloppenburg, Thomas Langer, Elena I Rugarli. J Clin Invest 2012
Eva R Almajan, Ricarda Richter, Lars Paeger, Paola Martinelli, Esther Barth, Thorsten Decker, Nils-Göran Larsson, Peter Kloppenburg, Thomas Langer, Elena I Rugarli. J Clin Invest 2012
26
OPA1 processing controls mitochondrial fusion and is regulated by mRNA splicing, membrane potential, and Yme1L.
Zhiyin Song, Hsiuchen Chen, Maja Fiket, Christiane Alexander, David C Chan. J Cell Biol 2007
Zhiyin Song, Hsiuchen Chen, Maja Fiket, Christiane Alexander, David C Chan. J Cell Biol 2007
26
Mitochondrial diseases: the contribution of organelle stress responses to pathology.
Anu Suomalainen, Brendan J Battersby. Nat Rev Mol Cell Biol 2018
Anu Suomalainen, Brendan J Battersby. Nat Rev Mol Cell Biol 2018
20
OPA1 processing in cell death and disease - the long and short of it.
Thomas MacVicar, Thomas Langer. J Cell Sci 2016
Thomas MacVicar, Thomas Langer. J Cell Sci 2016
20
OPA1 controls apoptotic cristae remodeling independently from mitochondrial fusion.
Christian Frezza, Sara Cipolat, Olga Martins de Brito, Massimo Micaroni, Galina V Beznoussenko, Tomasz Rudka, Davide Bartoli, Roman S Polishuck, Nika N Danial, Bart De Strooper,[...]. Cell 2006
Christian Frezza, Sara Cipolat, Olga Martins de Brito, Massimo Micaroni, Galina V Beznoussenko, Tomasz Rudka, Davide Bartoli, Roman S Polishuck, Nika N Danial, Bart De Strooper,[...]. Cell 2006
20
Stress-induced OMA1 activation and autocatalytic turnover regulate OPA1-dependent mitochondrial dynamics.
Michael J Baker, Philipp A Lampe, Diana Stojanovski, Anne Korwitz, Ruchika Anand, Takashi Tatsuta, Thomas Langer. EMBO J 2014
Michael J Baker, Philipp A Lampe, Diana Stojanovski, Anne Korwitz, Ruchika Anand, Takashi Tatsuta, Thomas Langer. EMBO J 2014
20
Clonal expansion of secondary mitochondrial DNA deletions associated with spinocerebellar ataxia type 28.
Gráinne S Gorman, Gerald Pfeffer, Helen Griffin, Emma L Blakely, Marzena Kurzawa-Akanbi, Jessica Gabriel, Kamil Sitarz, Mark Roberts, Benedikt Schoser, Angela Pyle,[...]. JAMA Neurol 2015
Gráinne S Gorman, Gerald Pfeffer, Helen Griffin, Emma L Blakely, Marzena Kurzawa-Akanbi, Jessica Gabriel, Kamil Sitarz, Mark Roberts, Benedikt Schoser, Angela Pyle,[...]. JAMA Neurol 2015
20
Regulation of mitochondrial morphology through proteolytic cleavage of OPA1.
Naotada Ishihara, Yuu Fujita, Toshihiko Oka, Katsuyoshi Mihara. EMBO J 2006
Naotada Ishihara, Yuu Fujita, Toshihiko Oka, Katsuyoshi Mihara. EMBO J 2006
20
Regulation of OPA1 processing and mitochondrial fusion by m-AAA protease isoenzymes and OMA1.
Sarah Ehses, Ines Raschke, Giuseppe Mancuso, Andrea Bernacchia, Stefan Geimer, Daniel Tondera, Jean-Claude Martinou, Benedikt Westermann, Elena I Rugarli, Thomas Langer. J Cell Biol 2009
Sarah Ehses, Ines Raschke, Giuseppe Mancuso, Andrea Bernacchia, Stefan Geimer, Daniel Tondera, Jean-Claude Martinou, Benedikt Westermann, Elena I Rugarli, Thomas Langer. J Cell Biol 2009
20
Early complex I assembly defects result in rapid turnover of the ND1 subunit.
Olga Zurita Rendón, Eric A Shoubridge. Hum Mol Genet 2012
Olga Zurita Rendón, Eric A Shoubridge. Hum Mol Genet 2012
20
Organization and Regulation of Mitochondrial Protein Synthesis.
Martin Ott, Alexey Amunts, Alan Brown. Annu Rev Biochem 2016
Martin Ott, Alexey Amunts, Alan Brown. Annu Rev Biochem 2016
20
Inducible proteolytic inactivation of OPA1 mediated by the OMA1 protease in mammalian cells.
Brian Head, Lorena Griparic, Mandana Amiri, Shilpa Gandre-Babbe, Alexander M van der Bliek. J Cell Biol 2009
Brian Head, Lorena Griparic, Mandana Amiri, Shilpa Gandre-Babbe, Alexander M van der Bliek. J Cell Biol 2009
20
The YTA10-12 complex, an AAA protease with chaperone-like activity in the inner membrane of mitochondria.
H Arlt, R Tauer, H Feldmann, W Neupert, T Langer. Cell 1996
H Arlt, R Tauer, H Feldmann, W Neupert, T Langer. Cell 1996
20
Nonsense mutations in the COX1 subunit impair the stability of respiratory chain complexes rather than their assembly.
Hue-Tran Hornig-Do, Takashi Tatsuta, Angela Buckermann, Maria Bust, Gittan Kollberg, Agnes Rötig, Martin Hellmich, Leo Nijtmans, Rudolf J Wiesner. EMBO J 2012
Hue-Tran Hornig-Do, Takashi Tatsuta, Angela Buckermann, Maria Bust, Gittan Kollberg, Agnes Rötig, Martin Hellmich, Leo Nijtmans, Rudolf J Wiesner. EMBO J 2012
20
Prohibitins control cell proliferation and apoptosis by regulating OPA1-dependent cristae morphogenesis in mitochondria.
Carsten Merkwirth, Sascha Dargazanli, Takashi Tatsuta, Stefan Geimer, Beatrix Löwer, F Thomas Wunderlich, Jürgen-Christoph von Kleist-Retzow, Ari Waisman, Benedikt Westermann, Thomas Langer. Genes Dev 2008
Carsten Merkwirth, Sascha Dargazanli, Takashi Tatsuta, Stefan Geimer, Beatrix Löwer, F Thomas Wunderlich, Jürgen-Christoph von Kleist-Retzow, Ari Waisman, Benedikt Westermann, Thomas Langer. Genes Dev 2008
20
AAA+ proteases: ATP-fueled machines of protein destruction.
Robert T Sauer, Tania A Baker. Annu Rev Biochem 2011
Robert T Sauer, Tania A Baker. Annu Rev Biochem 2011
20
Spastic paraplegia and OXPHOS impairment caused by mutations in paraplegin, a nuclear-encoded mitochondrial metalloprotease.
G Casari, M De Fusco, S Ciarmatori, M Zeviani, M Mora, P Fernandez, G De Michele, A Filla, S Cocozza, R Marconi,[...]. Cell 1998
G Casari, M De Fusco, S Ciarmatori, M Zeviani, M Mora, P Fernandez, G De Michele, A Filla, S Cocozza, R Marconi,[...]. Cell 1998
20
Non-syndromic isolated dominant optic atrophy caused by the p.R468C mutation in the AFG3 like matrix AAA peptidase subunit 2 gene.
Davide Colavito, Veronica Maritan, Agnese Suppiej, Elda Del Giudice, Monica Mazzarolo, Stefania Miotto, Sofia Farina, Maurizio Dalle Carbonare, Stefano Piermarocchi, Alberta Leon. Biomed Rep 2017
Davide Colavito, Veronica Maritan, Agnese Suppiej, Elda Del Giudice, Monica Mazzarolo, Stefania Miotto, Sofia Farina, Maurizio Dalle Carbonare, Stefano Piermarocchi, Alberta Leon. Biomed Rep 2017
25
Variable and tissue-specific subunit composition of mitochondrial m-AAA protease complexes linked to hereditary spastic paraplegia.
Mirko Koppen, Metodi D Metodiev, Giorgio Casari, Elena I Rugarli, Thomas Langer. Mol Cell Biol 2007
Mirko Koppen, Metodi D Metodiev, Giorgio Casari, Elena I Rugarli, Thomas Langer. Mol Cell Biol 2007
20
Membrane protein degradation by AAA proteases in mitochondria: extraction of substrates from either membrane surface.
K Leonhard, B Guiard, G Pellecchia, A Tzagoloff, W Neupert, T Langer. Mol Cell 2000
K Leonhard, B Guiard, G Pellecchia, A Tzagoloff, W Neupert, T Langer. Mol Cell 2000
20
Mitochondrial stress response triggered by defects in protein synthesis quality control.
Uwe Richter, Kah Ying Ng, Fumi Suomi, Paula Marttinen, Taina Turunen, Christopher Jackson, Anu Suomalainen, Helena Vihinen, Eija Jokitalo, Tuula A Nyman,[...]. Life Sci Alliance 2019
Uwe Richter, Kah Ying Ng, Fumi Suomi, Paula Marttinen, Taina Turunen, Christopher Jackson, Anu Suomalainen, Helena Vihinen, Eija Jokitalo, Tuula A Nyman,[...]. Life Sci Alliance 2019
20
Unique Structural Features of the Mitochondrial AAA+ Protease AFG3L2 Reveal the Molecular Basis for Activity in Health and Disease.
Cristina Puchades, Bojian Ding, Albert Song, R Luke Wiseman, Gabriel C Lander, Steven E Glynn. Mol Cell 2019
Cristina Puchades, Bojian Ding, Albert Song, R Luke Wiseman, Gabriel C Lander, Steven E Glynn. Mol Cell 2019
20
The membrane scaffold SLP2 anchors a proteolytic hub in mitochondria containing PARL and the i-AAA protease YME1L.
Timothy Wai, Shotaro Saita, Hendrik Nolte, Sebastian Müller, Tim König, Ricarda Richter-Dennerlein, Hans-Georg Sprenger, Joaquin Madrenas, Mareike Mühlmeister, Ulrich Brandt,[...]. EMBO Rep 2016
Timothy Wai, Shotaro Saita, Hendrik Nolte, Sebastian Müller, Tim König, Ricarda Richter-Dennerlein, Hans-Georg Sprenger, Joaquin Madrenas, Mareike Mühlmeister, Ulrich Brandt,[...]. EMBO Rep 2016
13
Ribosome. The structure of the human mitochondrial ribosome.
Alexey Amunts, Alan Brown, Jaan Toots, Sjors H W Scheres, V Ramakrishnan. Science 2015
Alexey Amunts, Alan Brown, Jaan Toots, Sjors H W Scheres, V Ramakrishnan. Science 2015
13
Loss of OMA1 delays neurodegeneration by preventing stress-induced OPA1 processing in mitochondria.
Anne Korwitz, Carsten Merkwirth, Ricarda Richter-Dennerlein, Simon E Tröder, Hans-Georg Sprenger, Pedro M Quirós, Carlos López-Otín, Elena I Rugarli, Thomas Langer. J Cell Biol 2016
Anne Korwitz, Carsten Merkwirth, Ricarda Richter-Dennerlein, Simon E Tröder, Hans-Georg Sprenger, Pedro M Quirós, Carlos López-Otín, Elena I Rugarli, Thomas Langer. J Cell Biol 2016
13
Loss of the m-AAA protease subunit AFG₃L₂ causes mitochondrial transport defects and tau hyperphosphorylation.
Arun Kumar Kondadi, Shuaiyu Wang, Sara Montagner, Nikolay Kladt, Anne Korwitz, Paola Martinelli, David Herholz, Michael J Baker, Astrid C Schauss, Thomas Langer,[...]. EMBO J 2014
Arun Kumar Kondadi, Shuaiyu Wang, Sara Montagner, Nikolay Kladt, Anne Korwitz, Paola Martinelli, David Herholz, Michael J Baker, Astrid C Schauss, Thomas Langer,[...]. EMBO J 2014
13
Missense mutations in the AFG3L2 proteolytic domain account for ∼1.5% of European autosomal dominant cerebellar ataxias.
Claudia Cagnoli, Giovanni Stevanin, Alessandro Brussino, Marco Barberis, Cecilia Mancini, Russell L Margolis, Susan E Holmes, Marcello Nobili, Sylvie Forlani, Sergio Padovan,[...]. Hum Mutat 2010
Claudia Cagnoli, Giovanni Stevanin, Alessandro Brussino, Marco Barberis, Cecilia Mancini, Russell L Margolis, Susan E Holmes, Marcello Nobili, Sylvie Forlani, Sergio Padovan,[...]. Hum Mutat 2010
13
A mitochondrial DNA microdeletion in a newborn girl with transient lactic acidosis.
S Seneca, M Abramowicz, W Lissens, M F Muller, E Vamos, L de Meirleir. J Inherit Metab Dis 1996
S Seneca, M Abramowicz, W Lissens, M F Muller, E Vamos, L de Meirleir. J Inherit Metab Dis 1996
13
Structure and conformational states of the bovine mitochondrial ATP synthase by cryo-EM.
Anna Zhou, Alexis Rohou, Daniel G Schep, John V Bason, Martin G Montgomery, John E Walker, Nikolaus Grigorieff, John L Rubinstein. Elife 2015
Anna Zhou, Alexis Rohou, Daniel G Schep, John V Bason, Martin G Montgomery, John E Walker, Nikolaus Grigorieff, John L Rubinstein. Elife 2015
13
New roles for mitochondrial proteases in health, ageing and disease.
Pedro M Quirós, Thomas Langer, Carlos López-Otín. Nat Rev Mol Cell Biol 2015
Pedro M Quirós, Thomas Langer, Carlos López-Otín. Nat Rev Mol Cell Biol 2015
13
Formation of cristae and crista junctions in mitochondria depends on antagonism between Fcj1 and Su e/g.
Regina Rabl, Vincent Soubannier, Roland Scholz, Frank Vogel, Nadine Mendl, Andreja Vasiljev-Neumeyer, Christian Körner, Ravi Jagasia, Thomas Keil, Wolfgang Baumeister,[...]. J Cell Biol 2009
Regina Rabl, Vincent Soubannier, Roland Scholz, Frank Vogel, Nadine Mendl, Andreja Vasiljev-Neumeyer, Christian Körner, Ravi Jagasia, Thomas Keil, Wolfgang Baumeister,[...]. J Cell Biol 2009
13
Small molecule inhibition of apicomplexan FtsH1 disrupts plastid biogenesis in human pathogens.
Katherine Amberg-Johnson, Sanjay B Hari, Suresh M Ganesan, Hernan A Lorenzi, Robert T Sauer, Jacquin C Niles, Ellen Yeh. Elife 2017
Katherine Amberg-Johnson, Sanjay B Hari, Suresh M Ganesan, Hernan A Lorenzi, Robert T Sauer, Jacquin C Niles, Ellen Yeh. Elife 2017
13
RNA modification landscape of the human mitochondrial tRNALys regulates protein synthesis.
Uwe Richter, Molly E Evans, Wesley C Clark, Paula Marttinen, Eric A Shoubridge, Anu Suomalainen, Anna Wredenberg, Anna Wedell, Tao Pan, Brendan J Battersby. Nat Commun 2018
Uwe Richter, Molly E Evans, Wesley C Clark, Paula Marttinen, Eric A Shoubridge, Anu Suomalainen, Anna Wredenberg, Anna Wedell, Tao Pan, Brendan J Battersby. Nat Commun 2018
13
A mitochondrial ribosomal and RNA decay pathway blocks cell proliferation.
Uwe Richter, Taina Lahtinen, Paula Marttinen, Maarit Myöhänen, Dario Greco, Giuseppe Cannino, Howard T Jacobs, Niina Lietzén, Tuula A Nyman, Brendan J Battersby. Curr Biol 2013
Uwe Richter, Taina Lahtinen, Paula Marttinen, Maarit Myöhänen, Dario Greco, Giuseppe Cannino, Howard T Jacobs, Niina Lietzén, Tuula A Nyman, Brendan J Battersby. Curr Biol 2013
13
Ribosome. The complete structure of the 55S mammalian mitochondrial ribosome.
Basil J Greber, Philipp Bieri, Marc Leibundgut, Alexander Leitner, Ruedi Aebersold, Daniel Boehringer, Nenad Ban. Science 2015
Basil J Greber, Philipp Bieri, Marc Leibundgut, Alexander Leitner, Ruedi Aebersold, Daniel Boehringer, Nenad Ban. Science 2015
13
Axonal degeneration in paraplegin-deficient mice is associated with abnormal mitochondria and impairment of axonal transport.
Fatima Ferreirinha, Angelo Quattrini, Marinella Pirozzi, Valentina Valsecchi, Giorgia Dina, Vania Broccoli, Alberto Auricchio, Fiorella Piemonte, Giulia Tozzi, Laura Gaeta,[...]. J Clin Invest 2004
Fatima Ferreirinha, Angelo Quattrini, Marinella Pirozzi, Valentina Valsecchi, Giorgia Dina, Vania Broccoli, Alberto Auricchio, Fiorella Piemonte, Giulia Tozzi, Laura Gaeta,[...]. J Clin Invest 2004
13
The Sequence Alignment/Map format and SAMtools.
Heng Li, Bob Handsaker, Alec Wysoker, Tim Fennell, Jue Ruan, Nils Homer, Gabor Marth, Goncalo Abecasis, Richard Durbin. Bioinformatics 2009
Heng Li, Bob Handsaker, Alec Wysoker, Tim Fennell, Jue Ruan, Nils Homer, Gabor Marth, Goncalo Abecasis, Richard Durbin. Bioinformatics 2009
13
The Neuropathology of Spinocerebellar Ataxia Type 3/Machado-Joseph Disease.
Arnulf H Koeppen. Adv Exp Med Biol 2018
Arnulf H Koeppen. Adv Exp Med Biol 2018
13
Antisense oligonucleotide therapy for spinocerebellar ataxia type 2.
Daniel R Scoles, Pratap Meera, Matthew D Schneider, Sharan Paul, Warunee Dansithong, Karla P Figueroa, Gene Hung, Frank Rigo, C Frank Bennett, Thomas S Otis,[...]. Nature 2017
Daniel R Scoles, Pratap Meera, Matthew D Schneider, Sharan Paul, Warunee Dansithong, Karla P Figueroa, Gene Hung, Frank Rigo, C Frank Bennett, Thomas S Otis,[...]. Nature 2017
13
Aminopyridines correct early dysfunction and delay neurodegeneration in a mouse model of spinocerebellar ataxia type 1.
Raphael Hourez, Laurent Servais, David Orduz, David Gall, Isabelle Millard, Alban de Kerchove d'Exaerde, Guy Cheron, Harry T Orr, Massimo Pandolfo, Serge N Schiffmann. J Neurosci 2011
Raphael Hourez, Laurent Servais, David Orduz, David Gall, Isabelle Millard, Alban de Kerchove d'Exaerde, Guy Cheron, Harry T Orr, Massimo Pandolfo, Serge N Schiffmann. J Neurosci 2011
13
Natural History of Spinocerebellar Ataxia Type 31: a 4-Year Prospective Study.
Katsuya Nakamura, Kunihiro Yoshida, Akira Matsushima, Yusaku Shimizu, Shunichi Sato, Hiroyuki Yahikozawa, Shinji Ohara, Masanobu Yazawa, Masao Ushiyama, Mitsuto Sato,[...]. Cerebellum 2017
Katsuya Nakamura, Kunihiro Yoshida, Akira Matsushima, Yusaku Shimizu, Shunichi Sato, Hiroyuki Yahikozawa, Shinji Ohara, Masanobu Yazawa, Masao Ushiyama, Mitsuto Sato,[...]. Cerebellum 2017
13
Familial hemiplegic migraine and episodic ataxia type-2 are caused by mutations in the Ca2+ channel gene CACNL1A4.
R A Ophoff, G M Terwindt, M N Vergouwe, R van Eijk, P J Oefner, S M Hoffman, J E Lamerdin, H W Mohrenweiser, D E Bulman, M Ferrari,[...]. Cell 1996
R A Ophoff, G M Terwindt, M N Vergouwe, R van Eijk, P J Oefner, S M Hoffman, J E Lamerdin, H W Mohrenweiser, D E Bulman, M Ferrari,[...]. Cell 1996
13
Autosomal dominant cerebellar ataxias: polyglutamine expansions and beyond.
Alexandra Durr. Lancet Neurol 2010
Alexandra Durr. Lancet Neurol 2010
13
4-aminopyridine reverses ataxia and cerebellar firing deficiency in a mouse model of spinocerebellar ataxia type 6.
Sriram Jayabal, Hui Ho Vanessa Chang, Kathleen E Cullen, Alanna J Watt. Sci Rep 2016
Sriram Jayabal, Hui Ho Vanessa Chang, Kathleen E Cullen, Alanna J Watt. Sci Rep 2016
13
Co-cited is the co-citation frequency, indicating how many articles cite the article together with the query article. Similarity is the co-citation as percentage of the times cited of the query article or the article in the search results, whichever is the lowest. These numbers are calculated for the last 100 citations when articles are cited more than 100 times.