2015
Benjamin Vitre, Andrew J. Holland, Anita Kulukian, Ofer Shoshani, Maretoshi Hirai, Yin Wang, Marcus Maldonado, Thomas Cho, Jihane Boubaker, Deborah A. Swing, Lino Tessarollo, Sylvia M. Evans, Elaine Fuchs, and Don W. Cleveland (2015). Chronic centrosome amplification without tumorigenesis. PNAS E6321-E6330
 
Marka van Blitterswijk, Tania F. Gendron, Matthew C. Baker, Mariely DeJesus-Hernandez, NiCole A. Finch, Patricia H. Brown, Lillian M. Daughrity, Melissa E. Murray, Michael G. Heckman, Jie Jiang, Clotilde Lagier-Tourenne, Dieter Edbauer, Don W. Cleveland, Keith A. Josephs, Joseph E. Parisi, David S. Knopman, Ronald C. Petersen, Leonard Petrucelli, Bradley F. Boeve, Neill R. Graff-Radford, Kevin B. Boylan, Dennis W. Dickson, Rosa Rademakers (2015). Novel clinical associations with specific C9ORF72 transcripts in patients with repeat expansions in C9ORF72. Acta Neuropathol (2015) 130:863–876
 
Shuying Sun, Ying Sun, Shuo-Chien Ling, Laura Ferraiuolo, Melissa McAlonis-Downes, Yiyang Zou, Kevin Drenner, Yin Wang, Dara Ditsworth, Seiya Tokunaga, Alex Kopelevich, Brian K. Kaspar, Clotilde Lagier-Tourenne, and Don W. Cleveland (2015). Translational profiling identifies a cascade of damage initiated in motor neurons and spreading to glia in mutant SOD1-mediated ALS. PNAS 112, E6993-E7002
 
Meghdad Rahdar, Moira A. McMahon, Thazha P. Prakash, Eric E. Swayze, C. Frank Bennett, and Don W. Cleveland (2015). Synthetic CRISPR RNA-Cas9–guided genome editing in human cells. PNAS 112, E7110-E7117
 
Jacqueline C Mitchell, Remy Constable, Eva So, Caroline Vance, Emma Scotter, Leanne Glover, Tibor Hortobagyi, Eveline S. Arnold, Shuo-Chien Ling, Melissa McAlonis, Sandrine Da Cruz, Magda Polymenidou, Lino Tessarolo,Don W Cleveland and Christopher E Shaw (2015). Wild type human TDP-43 potentiates ALSlinked mutant TDP-43 driven progressive motor and cortical neuron degeneration with pathological features of ALS. Acta Neuropathologica Communications 3, 36-51
 
Anita Kulukiana, Andrew J. Hollandb, Benjamin Vitrec, Shruti Naika, Don W. Cleveland, and Elaine Fuchsa (2015). Epidermal development, growth control, and homeostasis in the face of centrosome amplification. PNAS E6311-E6320
 
Andrew J. Hollanda, Rita M. Reisb, Sherry Niessend, Cláudia Pereirab, Douglas A. Andrese, H. Peter Spielmanne, Don W. Cleveland, Arshad Desaia, and Reto Gassmannb (2015). Preventing farnesylation of the dynein adaptor Spindly contributes to the mitotic defects caused by farnesyltransferase inhibitors. Mol Biol Cell 26, 1845-1856
 
Tania F. Gendron, Marka van Blitterswijk, Kevin F. Bieniek, Lillian M. Daughrity, Jie Jiang, Beth K. Rush, Otto Pedraza, John A. Lucas, Melissa E. Murray, Pamela Desaro, Amelia Robertson, Karen Overstreet, Colleen S. Thomas, Julia E. Crook, Monica Castanedes-Casey, Linda Rousseau, Keith A. Josephs, Joseph E. Parisi, David S. Knopman, Ronald C. Petersen, Bradley F. Boeve, Neill R. Graff-Radford, Rosa Rademakers, Clotilde Lagier-Tourenne, Dieter Edbauer, Don W. Cleveland, Dennis W. Dickson, Leonard Petrucelli, Kevin B. Boylan (2015). Cerebellar c9RAN proteins associate with clinical and neuropathological characteristics of C9ORF72 repeat expansion carriers. Acta Neuropathol (2015) 130:559–573
 
Stefano Bertuzzi and Don W. Cleveland (2015). The curious incident of the translational dog that didn't bark. Trends in Cell Biology April 2015, Vol. 25, No. 4
 
Caroline Topham, Anthony Tighe, Peter Ly, Ailsa Bennett, Olivia Sloss, Louisa Nelson, Rachel A. Ridgway,
David Huels, Samantha Littler, Claudia Schandl, Ying Sun, Beatrice Bechi, David J. Procter, Owen J. Sansom, Don W. Cleveland, and Stephen S. Taylor (2015). MYC Is a Major Determinant of Mitotic Cell Fate. Cancer Cell 28, 129–140
 
Daniele Fachinetti, Joo Seok Han, Moira A. McMahon, Peter Ly, Amira Abdullah, Alex J. Wong, andDon W. Cleveland (2015). DNA Sequence-Specific Binding of CENP-B Enhances the Fidelity of Human Centromere Function. Developmental Cell 33, 314–327
 
Adrian Israelson, Dara Ditsworth, Shuying Sun, SungWon Song, Jason Liang, Marian Hruska-Plochan,
Melissa McAlonis-Downes, Salah Abu-Hamad, Guy Zoltsman, Tom Shani, Marcus Maldonado, Anh Bui,
Michael Navarro, Huilin Zhou, Martin Marsala, Brian K. Kaspar, Sandrine Da Cruz, and Don W. Cleveland (2015). Macrophage Migration Inhibitory Factor as a Chaperone Inhibiting Accumulation of Misfolded SOD1. Neuron 86, 218–232.
 
Shuying Sun, Shuo-Chien Ling, Jinsong Qiu, Claudio P. Albuquerque, Yu Zhou, Seiya Tokunaga, Hairi Li, Haiyan Qiu, Anh Bui, Gene W. Yeo, Eric J. Huang, Kevin Eggan, Huilin Zhou, Xiang-Dong Fu, Clotilde Lagier-Tourenne & Don W. Cleveland (2015). ALS-causative mutations in FUS/TLS confer gain and loss of function by altered association with SMN and U1-snRNP. NATURE COMMUNICATIONS | 6:6171 | DOI: 10.1038/ncomms7171

Publications

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2016
Sandrine Da Cruz and Don W. Cleveland (2016). Disrupted nuclear importexport in neurodegeneration: Toxicity arises from amyloid-like protein accumulation in the cytoplasm, not the nucleus. Science 351, 125-126
 
Jie Jiang, Qiang Zhu, Tania F. Gendron, Shahram Saberi, Melissa McAlonis-Downes, Amanda Seelman, Jennifer E. Stauffer, Paymaan Jafar-nejad, Kevin Drenner, Derek Schulte, Seung Chun, Shuying Sun, Shuo-Chien Ling, Brian Myers, Jeffery Engelhardt, Melanie Katz, Michael Baughn, Oleksandr Platoshyn, Martin Marsala, Andy Watt, Charles J. Heyser, M. Colin Ard, Louis De Muynck, Lillian M. Daughrity, Deborah A. Swing, Lino Tessarollo, Chris J. Jung, Arnaud Delpoux, Daniel T. Utzschneider, Stephen M. Hedrick, Pieter J. de Jong, Dieter Edbauer, Philip Van Damme, Leonard Petrucelli, Christopher E. Shaw, C. Frank Bennett, Sandrine Da Cruz, John Ravits, Frank Rigo., Don W. Cleveland and Clotilde Lagier-Tourenne (2016).Gain of Toxicity from ALS/FTD-Linked Repeat Expansions in C9ORF72 Is Alleviated by Antisense Oligonucleotides TargetingGGGGCC-ContainingRNAs. Neuron 90, 1-6
 
Annelies Quaegebeur, Inmaculada Segura, Roberta Schmieder, Dries Verdegem, Ilaria Decimo, Francesco Bifari, Tom Dresselaers, Guy Eelen, Debapriva Ghosh, Shawn M. Davidson, Sandra Schoors, Dorien Broekaert, Bert Cruys, Kristof Govaerts, Carla De Legher, Ann Bouche´, Luc Schoonjans, Matt S. Ramer, Gene Hung, Goele Bossaert, Don W. Cleveland, Uwe Himmelreich, Thomas Voets, Robin Lemmens, C. Frank Bennett, Wim Robberecht, Katrien De Bock, Mieke Dewerchin, Bart Ghesquie`re, Sarah-Maria Fendt, and Peter Carmeliet (2016). Deletion or Inhibition of the Oxygen Sensor PHD1 Protects against Ischemic Stroke via Reprogramming of Neuronal Metabolism. Cell Metabolism 23, 280-291
 
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, Wen-Lang Lin, Aliesha Garrett, Jeannette N Stankowski, Lillian Daughrity, Jimei Tong, Emilie A Perkerson, Mei Yue, Jeannie Chew, Monica Castanedes Casey, Aishe Kurti, Zizhao S Wang, Amanda M Liesinger, Jeremy D Baker, Jie Jiang, Clotilde Lagier-Tourenne, Dieter Edbauer, Don W Cleveland, Rosa Rademakers, Kevin B Boylan, Guojun Bu, Christopher D Link, Chad A Dickey, Jeffrey D Rothstein, Dennis W Dickson, John D Fryer & Leonard Petrucelli (2016). C9ORF72 poly(GA) aggregates sequester and impair HR23 and nucleocytoplasmic transport proteins. Nature Neuroscience doi:10.1038/nn.4272
 
Sebastian Hoffmann, Marie Dumont, Viviana Barra, Peter Ly, Yael Nechemia-Arbely, Moira A. McMahon, Solene Herve, Don W. Cleveland, and Daniele Fachinetti (2016).  CENP-A Is Dispensable for Mitotic Centromere Function after Initial Centromere/Kinetochore Assembly.  Cell Reports 17, 2394–2404
 
Moira A. McMahon and Don W. Cleveland (2016). Gene-editing therapy for neurological disease. Nature Reviews Neurology 13:7–9 (2017)
 
Magdalini Polymenidou and Don W. Cleveland (2016). Biological Spectrum of Amyotrophic Lateral Sclerosis Prions. Cold Spring Harb Perspect Med doi: 10.1101/cshperspect.a024133
 
Tamara Seredenina, Zeynab Nayernia, Silvia Sorce, Ghassan J. Maghzal, Aleksandra Filippova, Shuo-Chien Ling, Olivier Basset, Olivier Plastre, Youssef Daali, Elisabeth J. Rushing, Maria T. Giordana, Don W. Cleveland, Adriano Aguzzi, Roland Stocker, Karl-Heinz Krause, Vincent Jaquet (2016). Evaluation of NADPH oxidases as drug targets in a mouse model of familial amyotrophic lateral sclerosis. Free Radical Biology and Medicine 97 (2016) 95–108
 
J. Paul Taylor, Robert H. Brown Jr & Don W. Cleveland (2016). Decoding ALS: from genes to mechanism. Nature 539, 197-206
2017
Qiaozhen Ye, Dong Hyun Kim, Ihsan Dereli, Scott C Rosenberg, Goetz Hagemann, Franz Herzog, Attila Tóth, Don W Cleveland & Kevin D Corbett (2017).  The AAA+ ATPase TRIP13 remodels HORMA domains through N-terminal engagement and unfolding.  The EMBO Journal (2017) Vol 36, No 16
 
Fen-Biao Gao, Joel D. Richter, and Don W. Cleveland (2017).  Rethinking Unconventional Translation in Neurodegeneration.  Cell 171, 994-1000
 
Peter Ly and Don W.Cleveland (2017). Rebuilding Chromosomes After Catastrophe: Emerging Mechanisms of Chromothripsis.  Trends in Cell Biology, 27: 917-930
 
Sandrine Da Cruz, Anh Bui, Shahram Saberi, Sandra K. Lee, Jennifer Stauffer, Melissa McAlonis-Downes, Derek Schulte, Donald P. Pizzo, Philippe A. Parone, Don W. Cleveland, John Ravits (2017).  Misfolded SOD1 is not a primary component of sporadic ALS.  Acta Neuropathol (2017) 134:97–111
 
Peter Ly, Levi S. Teitz, Dong H. Kim, Ofer Shoshani, Helen Skaletsky, Daniele Fachinetti, David C. Page and Don W. Cleveland (2017). Selective Y centromere inactivation triggers chromosome shattering in micronuclei and repair by non-homologous end joining.  NATURE CELL BIOLOGY VOLUME 19 | NUMBER 1 | JANUARY 2017
 
Peter Ly and Don W. Cleveland (2017).  Interrogating cell division errors using random and chromosome-specific missegregation approaches.  CELL CYCLE 2017, VOL. 16, NO. 13, 1252–1258
 
Dara Ditsworth, Marcus Maldonado, Melissa McAlonis-Downes, Shuying Sun, Amanda Seelman, Kevin Drenner, Eveline Arnold, Shuo-Chien Ling, Donald Pizzo, John Ravits, Don W. Cleveland*, and Sandrine Da Cruz1* (2017). Mutant TDP-43 within motor neurons drives disease onset but not progression in Amyotrophic Lateral Sclerosis. Acta Neuropathol, March 2017
 
Fatima Gasset-Rosa, Carlos Chillon-Marinas, Alexander Goginashvili, Ranjit Singh Atwal, Jonathan W. Artates, Ricardos Tabet, Vanessa C. Wheeler, Anne G. Bang, Don W. Cleveland* and Clotilde Lagier-Tourenne* (2017). Polyglutamine-Expanded Huntingtin Exacerbates Age-Related Disruption of Nuclear Integrity and Nucleocytoplasmic Transport. Neuron 94, 48–57, April 5, 2017
 
Lucie Y. Guo, Praveen Kumar Allu, Levani Zandarashvili, Kara L. McKinley, Nikolina Sekulic, Jennine M. Dawicki-McKenna, Daniele Fachinetti, Glennis A. Logsdon, Ryan M. Jamiolkowski, Don W. Cleveland, Iain M. Cheeseman & Ben E. Black (2017).  Centromeres are maintained by fastening CENP-A to DNA and directing an arginine anchordependent nucleosome transition.  NATURE COMMUNICATIONS | 8:15775
 
Michelle S. Levine, Bjorn Bakker, Bram Boeckx, Julia Moyett, James Lu, Benjamin Vitre, Diana C. Spierings, Peter M. Lansdorp, Don W. Cleveland, Diether Lambrechts, Floris Foijer, and Andrew J. Holland* (2017).Centrosome Amplification Is Sufficient to Promote Spontaneous Tumorigenesis in Mammals. Developmental Cell 40, 313–322, February 6, 2017
 
Moira A. McMahon and Don W. Cleveland (2017). Gene-editing therapy for neurological disease. Nature rev Neurol 13, 7-9
 
Yael Nechemia-Arbely, Daniele Fachinetti, Karen H. Miga, Nikolina Sekulic, Gautam V. Soni, Dong Hyun Kim, Adeline K. Wong, Ah Young Lee, Kristen Nguyen, Cees Dekker, Bing Ren, Ben E. Black, and Don W. Cleveland (2017) Human centromeric CENP-A chromatin is a homotypic, octameric nucleosome at all cell cycle points. J. Cell Biol. Vol. 216 No. 3 607–621
 
Magdalini Polymenidou and Don W. Cleveland (2017).Biological Spectrum of Amyotrophic Lateral Sclerosis Prions. Cold Spring Harb Perspect Med.
More Information on PubMed
Bakhoum SF, Ngo B, Laughney AM, Cavallo JA, Murphy CJ, Ly P, Shah P, Sriram RK, Watkins TBK, Taunk NK, Duran M, Pauli C, Shaw C, Chadalavada K, Rajasekhar VK, Genovese G, Venkatesan S, Birkbak NJ, McGranahan N, Lundquist M, LaPlant Q, Healey JH, Elemento O, Chung CH, Lee NY, Imielenski M, Nanjangud G, Pe’er D, Cleveland DW, Powell SN, Lammerding J, Swanton C, and Cantley LC (2018).  Chromosomal instability drives metastasis through a cytosolic DNA response.  Nature (2018) 553, 467-475
 
Weiwei Cheng, Shaopeng Wang, Alexander A. Mestre, Chenglai Fu, Andres Makarem, Fengfan Xian, Lindsey R. Hayes, Rodrigo Lopez-Gonzalez, Kevin Drenner, Jie Jiang, Don W. Cleveland & Shuying Sun (2018).  C9ORF72 GGGGCC repeat-associated non-AUG translation is upregulated by stress through eIF2a phosphorylation.  Nature Communications (2018) 9:51
 
Nikita Gudimchuk, Ekaterina V. Tarasovetc, Vadim Mustyatsa, Alexei L. Drobyshev, Benjamin Vitre, Don W. Cleveland, Fazly I. Ataullakhanov, and Ekaterina L. Grishchuk (2018). Probing Mitotic CENP-E Kinesin with the Tethered Cargo Motion Assay and Laser Tweezers. Biophysical Journal 114, 2640–2652, June 5, 2018
 
Moira A. McMahon, Thazha P. Prakash, Don W. Cleveland, C. Frank Bennett, and Meghdad Rahdar (2018). Chemically Modified Cpf1-CRISPR RNAs Mediate Efficient Genome Editing in Mammalian Cells. Molecular Therapy Vol. 26 No 5 May 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, Maria J. Rodriguez, Don Pizzo, Don Cleveland, John Ravits (2018). 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. Acta Neuropathologica (2018) 135:459–474
2018