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Times Cited: 114
Times Cited: 114
Times Cited
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Similarity
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
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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
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C9orf72 repeat expansions cause neurodegeneration in Drosophila through arginine-rich proteins.
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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
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C9orf72 Dipeptide Repeats Impair the Assembly, Dynamics, and Function of Membrane-Less Organelles.
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Antisense proline-arginine RAN dipeptides linked to C9ORF72-ALS/FTD form toxic nuclear aggregates that initiate in vitro and in vivo neuronal death.
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GGGGCC repeat expansion in C9orf72 compromises nucleocytoplasmic transport.
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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
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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
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RAN proteins and RNA foci from antisense transcripts in C9ORF72 ALS and frontotemporal dementia.
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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
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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
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Phase Separation of C9orf72 Dipeptide Repeats Perturbs Stress Granule Dynamics.
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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
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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
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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
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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
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Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis.
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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
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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
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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
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Heterochromatin anomalies and double-stranded RNA accumulation underlie C9orf72 poly(PR) toxicity.
Yong-Jie Zhang, Lin Guo, Patrick K Gonzales, Tania F Gendron, Yanwei Wu, Karen Jansen-West, Aliesha D O'Raw, Sarah R Pickles, Mercedes Prudencio, Yari Carlomagno,[...]. Science 2019
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RNA toxicity from the ALS/FTD C9ORF72 expansion is mitigated by antisense intervention.
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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
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Poly-dipeptides encoded by the C9ORF72 repeats block global protein translation.
Kohsuke Kanekura, Takuya Yagi, Alexander J Cammack, Jana Mahadevan, Masahiko Kuroda, Matthew B Harms, Timothy M Miller, Fumihiko Urano. Hum Mol Genet 2016
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Neurodegeneration. C9ORF72 repeat expansions in mice cause TDP-43 pathology, neuronal loss, and behavioral deficits.
Jeannie Chew, Tania F Gendron, Mercedes Prudencio, Hiroki Sasaguri, Yong-Jie Zhang, Monica Castanedes-Casey, Chris W Lee, Karen Jansen-West, Aishe Kurti, Melissa E Murray,[...]. Science 2015
Jeannie Chew, Tania F Gendron, Mercedes Prudencio, Hiroki Sasaguri, Yong-Jie Zhang, Monica Castanedes-Casey, Chris W Lee, Karen Jansen-West, Aishe Kurti, Melissa E Murray,[...]. Science 2015
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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
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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
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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
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29
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
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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
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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
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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
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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
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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
30
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
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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
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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
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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
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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
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Stress Granule Assembly Disrupts Nucleocytoplasmic Transport.
Ke Zhang, J Gavin Daigle, Kathleen M Cunningham, Alyssa N Coyne, Kai Ruan, Jonathan C Grima, Kelly E Bowen, Harsh Wadhwa, Peiguo Yang, Frank Rigo,[...]. Cell 2018
Ke Zhang, J Gavin Daigle, Kathleen M Cunningham, Alyssa N Coyne, Kai Ruan, Jonathan C Grima, Kelly E Bowen, Harsh Wadhwa, Peiguo Yang, Frank Rigo,[...]. Cell 2018
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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
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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
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FTD/ALS-associated poly(GR) protein impairs the Notch pathway and is recruited by poly(GA) into cytoplasmic inclusions.
Dejun Yang, Abbas Abdallah, Zhaodong Li, Yubing Lu, Sandra Almeida, Fen-Biao Gao. Acta Neuropathol 2015
Dejun Yang, Abbas Abdallah, Zhaodong Li, Yubing Lu, Sandra Almeida, Fen-Biao Gao. Acta Neuropathol 2015
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Loss-of-function mutations in the C9ORF72 mouse ortholog cause fatal autoimmune disease.
Aaron Burberry, Naoki Suzuki, Jin-Yuan Wang, Rob Moccia, Daniel A Mordes, Morag H Stewart, Satomi Suzuki-Uematsu, Sulagna Ghosh, Ajay Singh, Florian T Merkle,[...]. Sci Transl Med 2016
Aaron Burberry, Naoki Suzuki, Jin-Yuan Wang, Rob Moccia, Daniel A Mordes, Morag H Stewart, Satomi Suzuki-Uematsu, Sulagna Ghosh, Ajay Singh, Florian T Merkle,[...]. Sci Transl Med 2016
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Proteomics and C9orf72 neuropathology identify ribosomes as poly-GR/PR interactors driving toxicity.
Hannelore Hartmann, Daniel Hornburg, Mareike Czuppa, Jakob Bader, Meike Michaelsen, Daniel Farny, Thomas Arzberger, Matthias Mann, Felix Meissner, Dieter Edbauer. Life Sci Alliance 2018
Hannelore Hartmann, Daniel Hornburg, Mareike Czuppa, Jakob Bader, Meike Michaelsen, Daniel Farny, Thomas Arzberger, Matthias Mann, Felix Meissner, Dieter Edbauer. Life Sci Alliance 2018
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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
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The Role of Dipeptide Repeats in C9ORF72-Related ALS-FTD.
Brian D Freibaum, J Paul Taylor. Front Mol Neurosci 2017
Brian D Freibaum, J Paul Taylor. Front Mol Neurosci 2017
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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
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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.