Brian D Freibaum, J Paul Taylor. Front Mol Neurosci 2017
Times Cited: 122
Times Cited: 122
Times Cited
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Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS.
Mariely DeJesus-Hernandez, Ian R Mackenzie, Bradley F Boeve, Adam L Boxer, Matt Baker, Nicola J Rutherford, Alexandra M Nicholson, NiCole A Finch, Heather Flynn, Jennifer Adamson,[...]. Neuron 2011
Mariely DeJesus-Hernandez, Ian R Mackenzie, Bradley F Boeve, Adam L Boxer, Matt Baker, Nicola J Rutherford, Alexandra M Nicholson, NiCole A Finch, Heather Flynn, Jennifer Adamson,[...]. Neuron 2011
75
A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD.
Alan E Renton, Elisa Majounie, Adrian Waite, Javier Simón-Sánchez, Sara Rollinson, J Raphael Gibbs, Jennifer C Schymick, Hannu Laaksovirta, John C van Swieten, Liisa Myllykangas,[...]. Neuron 2011
Alan E Renton, Elisa Majounie, Adrian Waite, Javier Simón-Sánchez, Sara Rollinson, J Raphael Gibbs, Jennifer C Schymick, Hannu Laaksovirta, John C van Swieten, Liisa Myllykangas,[...]. Neuron 2011
71
Unconventional translation of C9ORF72 GGGGCC expansion generates insoluble polypeptides specific to c9FTD/ALS.
Peter E A Ash, Kevin F Bieniek, Tania F Gendron, Thomas Caulfield, Wen-Lang Lin, Mariely Dejesus-Hernandez, Marka M van Blitterswijk, Karen Jansen-West, Joseph W Paul, Rosa Rademakers,[...]. Neuron 2013
Peter E A Ash, Kevin F Bieniek, Tania F Gendron, Thomas Caulfield, Wen-Lang Lin, Mariely Dejesus-Hernandez, Marka M van Blitterswijk, Karen Jansen-West, Joseph W Paul, Rosa Rademakers,[...]. Neuron 2013
52
The C9orf72 GGGGCC repeat is translated into aggregating dipeptide-repeat proteins in FTLD/ALS.
Kohji Mori, Shih-Ming Weng, Thomas Arzberger, Stephanie May, Kristin Rentzsch, Elisabeth Kremmer, Bettina Schmid, Hans A Kretzschmar, Marc Cruts, Christine Van Broeckhoven,[...]. Science 2013
Kohji Mori, Shih-Ming Weng, Thomas Arzberger, Stephanie May, Kristin Rentzsch, Elisabeth Kremmer, Bettina Schmid, Hans A Kretzschmar, Marc Cruts, Christine Van Broeckhoven,[...]. Science 2013
47
Antisense transcripts of the expanded C9ORF72 hexanucleotide repeat form nuclear RNA foci and undergo repeat-associated non-ATG translation in c9FTD/ALS.
Tania F Gendron, Kevin F Bieniek, Yong-Jie Zhang, Karen Jansen-West, Peter E A Ash, Thomas Caulfield, Lillian Daughrity, Judith H Dunmore, Monica Castanedes-Casey, Jeannie Chew,[...]. Acta Neuropathol 2013
Tania F Gendron, Kevin F Bieniek, Yong-Jie Zhang, Karen Jansen-West, Peter E A Ash, Thomas Caulfield, Lillian Daughrity, Judith H Dunmore, Monica Castanedes-Casey, Jeannie Chew,[...]. Acta Neuropathol 2013
45
RAN proteins and RNA foci from antisense transcripts in C9ORF72 ALS and frontotemporal dementia.
Tao Zu, Yuanjing Liu, Monica Bañez-Coronel, Tammy Reid, Olga Pletnikova, Jada Lewis, Timothy M Miller, Matthew B Harms, Annet E Falchook, S H Subramony,[...]. Proc Natl Acad Sci U S A 2013
Tao Zu, Yuanjing Liu, Monica Bañez-Coronel, Tammy Reid, Olga Pletnikova, Jada Lewis, Timothy M Miller, Matthew B Harms, Annet E Falchook, S H Subramony,[...]. Proc Natl Acad Sci U S A 2013
44
The C9orf72 repeat expansion disrupts nucleocytoplasmic transport.
Ke Zhang, Christopher J Donnelly, Aaron R Haeusler, Jonathan C Grima, James B Machamer, Peter Steinwald, Elizabeth L Daley, Sean J Miller, Kathleen M Cunningham, Svetlana Vidensky,[...]. Nature 2015
Ke Zhang, Christopher J Donnelly, Aaron R Haeusler, Jonathan C Grima, James B Machamer, Peter Steinwald, Elizabeth L Daley, Sean J Miller, Kathleen M Cunningham, Svetlana Vidensky,[...]. Nature 2015
44
GGGGCC repeat expansion in C9orf72 compromises nucleocytoplasmic transport.
Brian D Freibaum, Yubing Lu, Rodrigo Lopez-Gonzalez, Nam Chul Kim, Sandra Almeida, Kyung-Ha Lee, Nisha Badders, Marc Valentine, Bruce L Miller, Philip C Wong,[...]. Nature 2015
Brian D Freibaum, Yubing Lu, Rodrigo Lopez-Gonzalez, Nam Chul Kim, Sandra Almeida, Kyung-Ha Lee, Nisha Badders, Marc Valentine, Bruce L Miller, Philip C Wong,[...]. Nature 2015
43
C9orf72 Dipeptide Repeats Impair the Assembly, Dynamics, and Function of Membrane-Less Organelles.
Kyung-Ha Lee, Peipei Zhang, Hong Joo Kim, Diana M Mitrea, Mohona Sarkar, Brian D Freibaum, Jaclyn Cika, Maura Coughlin, James Messing, Amandine Molliex,[...]. Cell 2016
Kyung-Ha Lee, Peipei Zhang, Hong Joo Kim, Diana M Mitrea, Mohona Sarkar, Brian D Freibaum, Jaclyn Cika, Maura Coughlin, James Messing, Amandine Molliex,[...]. Cell 2016
40
Bidirectional transcripts of the expanded C9orf72 hexanucleotide repeat are translated into aggregating dipeptide repeat proteins.
Kohji Mori, Thomas Arzberger, Friedrich A Grässer, Ilse Gijselinck, Stephanie May, Kristin Rentzsch, Shih-Ming Weng, Martin H Schludi, Julie van der Zee, Marc Cruts,[...]. Acta Neuropathol 2013
Kohji Mori, Thomas Arzberger, Friedrich A Grässer, Ilse Gijselinck, Stephanie May, Kristin Rentzsch, Shih-Ming Weng, Martin H Schludi, Julie van der Zee, Marc Cruts,[...]. Acta Neuropathol 2013
38
Antisense proline-arginine RAN dipeptides linked to C9ORF72-ALS/FTD form toxic nuclear aggregates that initiate in vitro and in vivo neuronal death.
Xinmei Wen, Wenzhi Tan, Thomas Westergard, Karthik Krishnamurthy, Shashirekha S Markandaiah, Yingxiao Shi, Shaoyu Lin, Neil A Shneider, John Monaghan, Udai B Pandey,[...]. Neuron 2014
Xinmei Wen, Wenzhi Tan, Thomas Westergard, Karthik Krishnamurthy, Shashirekha S Markandaiah, Yingxiao Shi, Shaoyu Lin, Neil A Shneider, John Monaghan, Udai B Pandey,[...]. Neuron 2014
38
Poly-dipeptides encoded by the C9orf72 repeats bind nucleoli, impede RNA biogenesis, and kill cells.
Ilmin Kwon, Siheng Xiang, Masato Kato, Leeju Wu, Pano Theodoropoulos, Tao Wang, Jiwoong Kim, Jonghyun Yun, Yang Xie, Steven L McKnight. Science 2014
Ilmin Kwon, Siheng Xiang, Masato Kato, Leeju Wu, Pano Theodoropoulos, Tao Wang, Jiwoong Kim, Jonghyun Yun, Yang Xie, Steven L McKnight. Science 2014
37
C9orf72 repeat expansions cause neurodegeneration in Drosophila through arginine-rich proteins.
Sarah Mizielinska, Sebastian Grönke, Teresa Niccoli, Charlotte E Ridler, Emma L Clayton, Anny Devoy, Thomas Moens, Frances E Norona, Ione O C Woollacott, Julian Pietrzyk,[...]. Science 2014
Sarah Mizielinska, Sebastian Grönke, Teresa Niccoli, Charlotte E Ridler, Emma L Clayton, Anny Devoy, Thomas Moens, Frances E Norona, Ione O C Woollacott, Julian Pietrzyk,[...]. Science 2014
36
Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis.
Manuela Neumann, Deepak M Sampathu, Linda K Kwong, Adam C Truax, Matthew C Micsenyi, Thomas T Chou, Jennifer Bruce, Theresa Schuck, Murray Grossman, Christopher M Clark,[...]. Science 2006
Manuela Neumann, Deepak M Sampathu, Linda K Kwong, Adam C Truax, Matthew C Micsenyi, Thomas T Chou, Jennifer Bruce, Theresa Schuck, Murray Grossman, Christopher M Clark,[...]. Science 2006
36
Modifiers of C9orf72 dipeptide repeat toxicity connect nucleocytoplasmic transport defects to FTD/ALS.
Ana Jovičić, Jerome Mertens, Steven Boeynaems, Elke Bogaert, Noori Chai, Shizuka B Yamada, Joseph W Paul, Shuying Sun, Joseph R Herdy, Gregor Bieri,[...]. Nat Neurosci 2015
Ana Jovičić, Jerome Mertens, Steven Boeynaems, Elke Bogaert, Noori Chai, Shizuka B Yamada, Joseph W Paul, Shuying Sun, Joseph R Herdy, Gregor Bieri,[...]. Nat Neurosci 2015
35
C9orf72 nucleotide repeat structures initiate molecular cascades of disease.
Aaron R Haeusler, Christopher J Donnelly, Goran Periz, Eric A J Simko, Patrick G Shaw, Min-Sik Kim, Nicholas J Maragakis, Juan C Troncoso, Akhilesh Pandey, Rita Sattler,[...]. Nature 2014
Aaron R Haeusler, Christopher J Donnelly, Goran Periz, Eric A J Simko, Patrick G Shaw, Min-Sik Kim, Nicholas J Maragakis, Juan C Troncoso, Akhilesh Pandey, Rita Sattler,[...]. Nature 2014
34
Decoding ALS: from genes to mechanism.
J Paul Taylor, Robert H Brown, Don W Cleveland. Nature 2016
J Paul Taylor, Robert H Brown, Don W Cleveland. Nature 2016
32
C9orf72 FTLD/ALS-associated Gly-Ala dipeptide repeat proteins cause neuronal toxicity and Unc119 sequestration.
Stephanie May, Daniel Hornburg, Martin H Schludi, Thomas Arzberger, Kristin Rentzsch, Benjamin M Schwenk, Friedrich A Grässer, Kohji Mori, Elisabeth Kremmer, Julia Banzhaf-Strathmann,[...]. Acta Neuropathol 2014
Stephanie May, Daniel Hornburg, Martin H Schludi, Thomas Arzberger, Kristin Rentzsch, Benjamin M Schwenk, Friedrich A Grässer, Kohji Mori, Elisabeth Kremmer, Julia Banzhaf-Strathmann,[...]. Acta Neuropathol 2014
31
RNA toxicity from the ALS/FTD C9ORF72 expansion is mitigated by antisense intervention.
Christopher J Donnelly, Ping-Wu Zhang, Jacqueline T Pham, Aaron R Haeusler, Nipun A Mistry, Svetlana Vidensky, Elizabeth L Daley, Erin M Poth, Benjamin Hoover, Daniel M Fines,[...]. Neuron 2013
Christopher J Donnelly, Ping-Wu Zhang, Jacqueline T Pham, Aaron R Haeusler, Nipun A Mistry, Svetlana Vidensky, Elizabeth L Daley, Erin M Poth, Benjamin Hoover, Daniel M Fines,[...]. Neuron 2013
30
Poly(GR) impairs protein translation and stress granule dynamics in C9orf72-associated frontotemporal dementia and amyotrophic lateral sclerosis.
Yong-Jie Zhang, Tania F Gendron, Mark T W Ebbert, Aliesha D O'Raw, Mei Yue, Karen Jansen-West, Xu Zhang, Mercedes Prudencio, Jeannie Chew, Casey N Cook,[...]. Nat Med 2018
Yong-Jie Zhang, Tania F Gendron, Mark T W Ebbert, Aliesha D O'Raw, Mei Yue, Karen Jansen-West, Xu Zhang, Mercedes Prudencio, Jeannie Chew, Casey N Cook,[...]. Nat Med 2018
29
Non-ATG-initiated translation directed by microsatellite expansions.
Tao Zu, Brian Gibbens, Noelle S Doty, Mário Gomes-Pereira, Aline Huguet, Matthew D Stone, Jamie Margolis, Mark Peterson, Todd W Markowski, Melissa A C Ingram,[...]. Proc Natl Acad Sci U S A 2011
Tao Zu, Brian Gibbens, Noelle S Doty, Mário Gomes-Pereira, Aline Huguet, Matthew D Stone, Jamie Margolis, Mark Peterson, Todd W Markowski, Melissa A C Ingram,[...]. Proc Natl Acad Sci U S A 2011
28
C9ORF72 poly(GA) aggregates sequester and impair HR23 and nucleocytoplasmic transport proteins.
Yong-Jie Zhang, Tania F Gendron, Jonathan C Grima, Hiroki Sasaguri, Karen Jansen-West, Ya-Fei Xu, Rebecca B Katzman, Jennifer Gass, Melissa E Murray, Mitsuru Shinohara,[...]. Nat Neurosci 2016
Yong-Jie Zhang, Tania F Gendron, Jonathan C Grima, Hiroki Sasaguri, Karen Jansen-West, Ya-Fei Xu, Rebecca B Katzman, Jennifer Gass, Melissa E Murray, Mitsuru Shinohara,[...]. Nat Neurosci 2016
28
Haploinsufficiency leads to neurodegeneration in C9ORF72 ALS/FTD human induced motor neurons.
Yingxiao Shi, Shaoyu Lin, Kim A Staats, Yichen Li, Wen-Hsuan Chang, Shu-Ting Hung, Eric Hendricks, Gabriel R Linares, Yaoming Wang, Esther Y Son,[...]. Nat Med 2018
Yingxiao Shi, Shaoyu Lin, Kim A Staats, Yichen Li, Wen-Hsuan Chang, Shu-Ting Hung, Eric Hendricks, Gabriel R Linares, Yaoming Wang, Esther Y Son,[...]. Nat Med 2018
27
Gain of Toxicity from ALS/FTD-Linked Repeat Expansions in C9ORF72 Is Alleviated by Antisense Oligonucleotides Targeting GGGGCC-Containing RNAs.
Jie Jiang, Qiang Zhu, Tania F Gendron, Shahram Saberi, Melissa McAlonis-Downes, Amanda Seelman, Jennifer E Stauffer, Paymaan Jafar-Nejad, Kevin Drenner, Derek Schulte,[...]. Neuron 2016
Jie Jiang, Qiang Zhu, Tania F Gendron, Shahram Saberi, Melissa McAlonis-Downes, Amanda Seelman, Jennifer E Stauffer, Paymaan Jafar-Nejad, Kevin Drenner, Derek Schulte,[...]. Neuron 2016
26
Drosophila screen connects nuclear transport genes to DPR pathology in c9ALS/FTD.
Steven Boeynaems, Elke Bogaert, Emiel Michiels, Ilse Gijselinck, Anne Sieben, Ana Jovičić, Greet De Baets, Wendy Scheveneels, Jolien Steyaert, Ivy Cuijt,[...]. Sci Rep 2016
Steven Boeynaems, Elke Bogaert, Emiel Michiels, Ilse Gijselinck, Anne Sieben, Ana Jovičić, Greet De Baets, Wendy Scheveneels, Jolien Steyaert, Ivy Cuijt,[...]. Sci Rep 2016
26
Quantitative analysis and clinico-pathological correlations of different dipeptide repeat protein pathologies in C9ORF72 mutation carriers.
Ian R A Mackenzie, Petra Frick, Friedrich A Grässer, Tania F Gendron, Leonard Petrucelli, Neil R Cashman, Dieter Edbauer, Elisabeth Kremmer, Johannes Prudlo, Dirk Troost,[...]. Acta Neuropathol 2015
Ian R A Mackenzie, Petra Frick, Friedrich A Grässer, Tania F Gendron, Leonard Petrucelli, Neil R Cashman, Dieter Edbauer, Elisabeth Kremmer, Johannes Prudlo, Dirk Troost,[...]. Acta Neuropathol 2015
26
C9orf72-mediated ALS and FTD: multiple pathways to disease.
Rubika Balendra, Adrian M Isaacs. Nat Rev Neurol 2018
Rubika Balendra, Adrian M Isaacs. Nat Rev Neurol 2018
26
Targeting RNA foci in iPSC-derived motor neurons from ALS patients with a C9ORF72 repeat expansion.
Dhruv Sareen, Jacqueline G O'Rourke, Pratap Meera, A K M G Muhammad, Sharday Grant, Megan Simpkinson, Shaughn Bell, Sharon Carmona, Loren Ornelas, Anais Sahabian,[...]. Sci Transl Med 2013
Dhruv Sareen, Jacqueline G O'Rourke, Pratap Meera, A K M G Muhammad, Sharday Grant, Megan Simpkinson, Shaughn Bell, Sharon Carmona, Loren Ornelas, Anais Sahabian,[...]. Sci Transl Med 2013
26
Phase Separation of C9orf72 Dipeptide Repeats Perturbs Stress Granule Dynamics.
Steven Boeynaems, Elke Bogaert, Denes Kovacs, Albert Konijnenberg, Evy Timmerman, Alex Volkov, Mainak Guharoy, Mathias De Decker, Tom Jaspers, Veronica H Ryan,[...]. Mol Cell 2017
Steven Boeynaems, Elke Bogaert, Denes Kovacs, Albert Konijnenberg, Evy Timmerman, Alex Volkov, Mainak Guharoy, Mathias De Decker, Tom Jaspers, Veronica H Ryan,[...]. Mol Cell 2017
26
Hexanucleotide repeats in ALS/FTD form length-dependent RNA foci, sequester RNA binding proteins, and are neurotoxic.
Youn-Bok Lee, Han-Jou Chen, João N Peres, Jorge Gomez-Deza, Jan Attig, Maja Stalekar, Claire Troakes, Agnes L Nishimura, Emma L Scotter, Caroline Vance,[...]. Cell Rep 2013
Youn-Bok Lee, Han-Jou Chen, João N Peres, Jorge Gomez-Deza, Jan Attig, Maja Stalekar, Claire Troakes, Agnes L Nishimura, Emma L Scotter, Caroline Vance,[...]. Cell Rep 2013
25
Nucleolar stress and impaired stress granule formation contribute to C9orf72 RAN translation-induced cytotoxicity.
Zhouteng Tao, Hongfeng Wang, Qin Xia, Ke Li, Kai Li, Xiaogang Jiang, Guoqiang Xu, Guanghui Wang, Zheng Ying. Hum Mol Genet 2015
Zhouteng Tao, Hongfeng Wang, Qin Xia, Ke Li, Kai Li, Xiaogang Jiang, Guoqiang Xu, Guanghui Wang, Zheng Ying. Hum Mol Genet 2015
25
Targeted degradation of sense and antisense C9orf72 RNA foci as therapy for ALS and frontotemporal degeneration.
Clotilde Lagier-Tourenne, Michael Baughn, Frank Rigo, Shuying Sun, Patrick Liu, Hai-Ri Li, Jie Jiang, Andrew T Watt, Seung Chun, Melanie Katz,[...]. Proc Natl Acad Sci U S A 2013
Clotilde Lagier-Tourenne, Michael Baughn, Frank Rigo, Shuying Sun, Patrick Liu, Hai-Ri Li, Jie Jiang, Andrew T Watt, Seung Chun, Melanie Katz,[...]. Proc Natl Acad Sci U S A 2013
24
C9orf72 frontotemporal lobar degeneration is characterised by frequent neuronal sense and antisense RNA foci.
Sarah Mizielinska, Tammaryn Lashley, Frances E Norona, Emma L Clayton, Charlotte E Ridler, Pietro Fratta, Adrian M Isaacs. Acta Neuropathol 2013
Sarah Mizielinska, Tammaryn Lashley, Frances E Norona, Emma L Clayton, Charlotte E Ridler, Pietro Fratta, Adrian M Isaacs. Acta Neuropathol 2013
24
Sense-encoded poly-GR dipeptide repeat proteins correlate to neurodegeneration and uniquely co-localize with TDP-43 in dendrites of repeat-expanded C9orf72 amyotrophic lateral sclerosis.
Shahram Saberi, Jennifer E Stauffer, Jie Jiang, Sandra Diaz Garcia, Amy E Taylor, Derek Schulte, Takuya Ohkubo, Cheyenne L Schloffman, Marcus Maldonado, Michael Baughn,[...]. Acta Neuropathol 2018
Shahram Saberi, Jennifer E Stauffer, Jie Jiang, Sandra Diaz Garcia, Amy E Taylor, Derek Schulte, Takuya Ohkubo, Cheyenne L Schloffman, Marcus Maldonado, Michael Baughn,[...]. Acta Neuropathol 2018
25
Toxic PRn poly-dipeptides encoded by the C9orf72 repeat expansion block nuclear import and export.
Kevin Y Shi, Eiichiro Mori, Zehra F Nizami, Yi Lin, Masato Kato, Siheng Xiang, Leeju C Wu, Ming Ding, Yonghao Yu, Joseph G Gall,[...]. Proc Natl Acad Sci U S A 2017
Kevin Y Shi, Eiichiro Mori, Zehra F Nizami, Yi Lin, Masato Kato, Siheng Xiang, Leeju C Wu, Ming Ding, Yonghao Yu, Joseph G Gall,[...]. Proc Natl Acad Sci U S A 2017
24
Sequestration of multiple RNA recognition motif-containing proteins by C9orf72 repeat expansions.
Johnathan Cooper-Knock, Matthew J Walsh, Adrian Higginbottom, J Robin Highley, Mark J Dickman, Dieter Edbauer, Paul G Ince, Stephen B Wharton, Stuart A Wilson, Janine Kirby,[...]. Brain 2014
Johnathan Cooper-Knock, Matthew J Walsh, Adrian Higginbottom, J Robin Highley, Mark J Dickman, Dieter Edbauer, Paul G Ince, Stephen B Wharton, Stuart A Wilson, Janine Kirby,[...]. Brain 2014
22
Aggregation-prone c9FTD/ALS poly(GA) RAN-translated proteins cause neurotoxicity by inducing ER stress.
Yong-Jie Zhang, Karen Jansen-West, Ya-Fei Xu, Tania F Gendron, Kevin F Bieniek, Wen-Lang Lin, Hiroki Sasaguri, Thomas Caulfield, Jaime Hubbard, Lillian Daughrity,[...]. Acta Neuropathol 2014
Yong-Jie Zhang, Karen Jansen-West, Ya-Fei Xu, Tania F Gendron, Kevin F Bieniek, Wen-Lang Lin, Hiroki Sasaguri, Thomas Caulfield, Jaime Hubbard, Lillian Daughrity,[...]. Acta Neuropathol 2014
22
A C9orf72 promoter repeat expansion in a Flanders-Belgian cohort with disorders of the frontotemporal lobar degeneration-amyotrophic lateral sclerosis spectrum: a gene identification study.
Ilse Gijselinck, Tim Van Langenhove, Julie van der Zee, Kristel Sleegers, Stéphanie Philtjens, Gernot Kleinberger, Jonathan Janssens, Karolien Bettens, Caroline Van Cauwenberghe, Sandra Pereson,[...]. Lancet Neurol 2012
Ilse Gijselinck, Tim Van Langenhove, Julie van der Zee, Kristel Sleegers, Stéphanie Philtjens, Gernot Kleinberger, Jonathan Janssens, Karolien Bettens, Caroline Van Cauwenberghe, Sandra Pereson,[...]. Lancet Neurol 2012
21
Distribution of dipeptide repeat proteins in cellular models and C9orf72 mutation cases suggests link to transcriptional silencing.
Martin H Schludi, Stephanie May, Friedrich A Grässer, Kristin Rentzsch, Elisabeth Kremmer, Clemens Küpper, Thomas Klopstock, Thomas Arzberger, Dieter Edbauer. Acta Neuropathol 2015
Martin H Schludi, Stephanie May, Friedrich A Grässer, Kristin Rentzsch, Elisabeth Kremmer, Clemens Küpper, Thomas Klopstock, Thomas Arzberger, Dieter Edbauer. Acta Neuropathol 2015
21
Dipeptide repeat protein pathology in C9ORF72 mutation cases: clinico-pathological correlations.
Ian R Mackenzie, Thomas Arzberger, Elisabeth Kremmer, Dirk Troost, Stefan Lorenzl, Kohji Mori, Shih-Ming Weng, Christian Haass, Hans A Kretzschmar, Dieter Edbauer,[...]. Acta Neuropathol 2013
Ian R Mackenzie, Thomas Arzberger, Elisabeth Kremmer, Dirk Troost, Stefan Lorenzl, Kohji Mori, Shih-Ming Weng, Christian Haass, Hans A Kretzschmar, Dieter Edbauer,[...]. Acta Neuropathol 2013
21
Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasis.
Shuo-Chien Ling, Magdalini Polymenidou, Don W Cleveland. Neuron 2013
Shuo-Chien Ling, Magdalini Polymenidou, Don W Cleveland. Neuron 2013
20
C9orf72 is required for proper macrophage and microglial function in mice.
J G O'Rourke, L Bogdanik, A Yáñez, D Lall, A J Wolf, A K M G Muhammad, R Ho, S Carmona, J P Vit, J Zarrow,[...]. Science 2016
J G O'Rourke, L Bogdanik, A Yáñez, D Lall, A J Wolf, A K M G Muhammad, R Ho, S Carmona, J P Vit, J Zarrow,[...]. Science 2016
20
The C9ORF72 GGGGCC expansion forms RNA G-quadruplex inclusions and sequesters hnRNP H to disrupt splicing in ALS brains.
Erin G Conlon, Lei Lu, Aarti Sharma, Takashi Yamazaki, Timothy Tang, Neil A Shneider, James L Manley. Elife 2016
Erin G Conlon, Lei Lu, Aarti Sharma, Takashi Yamazaki, Timothy Tang, Neil A Shneider, James L Manley. Elife 2016
20
C9orf72 ablation in mice does not cause motor neuron degeneration or motor deficits.
Max Koppers, Anna M Blokhuis, Henk-Jan Westeneng, Margo L Terpstra, Caroline A C Zundel, Renata Vieira de Sá, Raymond D Schellevis, Adrian J Waite, Derek J Blake, Jan H Veldink,[...]. Ann Neurol 2015
Max Koppers, Anna M Blokhuis, Henk-Jan Westeneng, Margo L Terpstra, Caroline A C Zundel, Renata Vieira de Sá, Raymond D Schellevis, Adrian J Waite, Derek J Blake, Jan H Veldink,[...]. Ann Neurol 2015
20
Loss of C9ORF72 impairs autophagy and synergizes with polyQ Ataxin-2 to induce motor neuron dysfunction and cell death.
Chantal Sellier, Maria-Letizia Campanari, Camille Julie Corbier, Angeline Gaucherot, Isabelle Kolb-Cheynel, Mustapha Oulad-Abdelghani, Frank Ruffenach, Adeline Page, Sorana Ciura, Edor Kabashi,[...]. EMBO J 2016
Chantal Sellier, Maria-Letizia Campanari, Camille Julie Corbier, Angeline Gaucherot, Isabelle Kolb-Cheynel, Mustapha Oulad-Abdelghani, Frank Ruffenach, Adeline Page, Sorana Ciura, Edor Kabashi,[...]. EMBO J 2016
20
C9orf72 BAC Mouse Model with Motor Deficits and Neurodegenerative Features of ALS/FTD.
Yuanjing Liu, Amrutha Pattamatta, Tao Zu, Tammy Reid, Olgert Bardhi, David R Borchelt, Anthony T Yachnis, Laura P W Ranum. Neuron 2016
Yuanjing Liu, Amrutha Pattamatta, Tao Zu, Tammy Reid, Olgert Bardhi, David R Borchelt, Anthony T Yachnis, Laura P W Ranum. Neuron 2016
19
Poly(GR) in C9ORF72-Related ALS/FTD Compromises Mitochondrial Function and Increases Oxidative Stress and DNA Damage in iPSC-Derived Motor Neurons.
Rodrigo Lopez-Gonzalez, Yubing Lu, Tania F Gendron, Anna Karydas, Helene Tran, Dejun Yang, Leonard Petrucelli, Bruce L Miller, Sandra Almeida, Fen-Biao Gao. Neuron 2016
Rodrigo Lopez-Gonzalez, Yubing Lu, Tania F Gendron, Anna Karydas, Helene Tran, Dejun Yang, Leonard Petrucelli, Bruce L Miller, Sandra Almeida, Fen-Biao Gao. Neuron 2016
19
Toxic PR Poly-Dipeptides Encoded by the C9orf72 Repeat Expansion Target LC Domain Polymers.
Yi Lin, Eiichiro Mori, Masato Kato, Siheng Xiang, Leeju Wu, Ilmin Kwon, Steven L McKnight. Cell 2016
Yi Lin, Eiichiro Mori, Masato Kato, Siheng Xiang, Leeju Wu, Ilmin Kwon, Steven L McKnight. Cell 2016
19
Antisense RNA foci in the motor neurons of C9ORF72-ALS patients are associated with TDP-43 proteinopathy.
Johnathan Cooper-Knock, Adrian Higginbottom, Matthew J Stopford, J Robin Highley, Paul G Ince, Stephen B Wharton, Stuart Pickering-Brown, Janine Kirby, Guillaume M Hautbergue, Pamela J Shaw. Acta Neuropathol 2015
Johnathan Cooper-Knock, Adrian Higginbottom, Matthew J Stopford, J Robin Highley, Paul G Ince, Stephen B Wharton, Stuart Pickering-Brown, Janine Kirby, Guillaume M Hautbergue, Pamela J Shaw. Acta Neuropathol 2015
18
TDP-43 pathology disrupts nuclear pore complexes and nucleocytoplasmic transport in ALS/FTD.
Ching-Chieh Chou, Yi Zhang, Mfon E Umoh, Spencer W Vaughan, Ileana Lorenzini, Feilin Liu, Melissa Sayegh, Paul G Donlin-Asp, Yu Han Chen, Duc M Duong,[...]. Nat Neurosci 2018
Ching-Chieh Chou, Yi Zhang, Mfon E Umoh, Spencer W Vaughan, Ileana Lorenzini, Feilin Liu, Melissa Sayegh, Paul G Donlin-Asp, Yu Han Chen, Duc M Duong,[...]. Nat Neurosci 2018
18
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.