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
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
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
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
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
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,
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
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
2017
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
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
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
2018
Jone Lopez-Erauskin, Takahiro Tadokoro, Michael W. Baughn, Brian Myers, Melissa McAlonis-Downes, Carlos Chillon-Marinas,
Joshua N. Asiaban, Jonathan Artates, Anh T. Bui, Anne P. Vetto, Sandra K. Lee, Ai Vy Le, Ying Sun, Melanie Jambeau, Jihane Boubaker,
Deborah Swing, Jinsong Qiu, Geoffrey G. Hicks, Zhengyu Ouyang, Xiang-Dong Fu, Lino Tessarollo, Shuo-Chien Ling, Philippe A. Parone,
Christopher E. Shaw, Martin Marsala, Clotilde Lagier-Tourenne,
Don W. Cleveland, and Sandrine Da Cruz (2018).
ALS/FTD-Linked Mutation
in FUS Suppresses Intra-axonal Protein Synthesis and Drives Disease Without Nuclear Loss-of-Function of FUS. Neuron
Volume 100, Issue
4, 21 November 2018, Pages 816-830.e7
Craig L. Bennett, Somasish G. Dastidar, Shuo-Chien Ling, Bilal Malik, Travis Ashe, Mandheer
Wadhwa, Derek B. Miller, Changwoo Lee, Matthew B. Mitchell, Michael A. van Es, Christopher Grunseich, Yingzhang Chen, Bryce L. Sopher,
Linda Greensmith,
Don W. Cleveland, Albert R. La Spada (2018).
Senataxin mutations elicit motor neuron degeneration phenotypes and
yield TDP-43 mislocalization in ALS4 mice and human patients. Acta Neuropathologica (2018) 136:425–443
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
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
2019
Peter Ly, Simon F. Brunner, Ofer Shoshani, Dong Hyun Kim, Weijie Lan, Tatyana Pyntikova, Adrienne M. Flanagan, Sam Behjati,
David C. Page, Peter J. Campbell and
Don W. Cleveland (2019).
Chromosome segregation errors generate a diverse spectrum of simple
and complex genomic rearrangements. Nature Genetics 51, 705-715 (2019)
Ze’ev Melamed1, Jone López-Erauskin, Michael W. Baughn,
Ouyang Zhang, Kevin Drenner, Ying Sun, Fernande Freyermuth, Moira A. McMahon, Melinda S. Beccari, Jon W. Artates, Takuya Ohkubo, Maria
Rodriguez, Nianwei Lin, Dongmei Wu, C. Frank Bennett, Frank Rigo, Sandrine Da Cruz, John Ravits, Clotilde Lagier-Tourenne and
Don
W. Cleveland (2019).
Premature polyadenylation-mediated loss of stathmin-2 is a hallmark of TDP-43-dependent neurodegeneration. Nature
Neuroscience 22, 180-190 (2019)
Shuo-Chien Ling, Somasish Ghosh Dastidar, Seiya
Tokunaga, Wan Yun Ho, Kenneth Lim, Hristelina Ilieva, Philippe A Parone, Sheue-Houy Tyan, Tsemay M Tse, Jer-Cherng Chang, Oleksandr
Platoshyn, Ngoc B Bui, Anh Bui, Anne Vetto, Shuying Sun, Melissa McAlonis-Downes, Joo Seok Han, Debbie Swing, Katannya Kapeli, Gene
W Yeo, Lino Tessarollo, Martin Marsala, Christopher E Shaw, Greg Tucker-Kellogg, Albert R La Spada, Clotilde Lagier-Tourenne, Sandrine
Da Cruz,
Don W Cleveland (2019).
Overriding FUS autoregulation in mice triggers gain-of-toxic dysfunctions in RNA metabolism and autophagy-lysosome
axis. eLife 2019;8:e40811
Fatima Gasset-Rosa, Shan Lu, Haiyang Yu, Cong Chen, Ze’ev Melamed, Lin Guo, James Shorter, Sandrine
Da Cruz, and
Don W. Cleveland (2019).
Cytoplasmic TDP-43 De-mixing Independent of Stress Granules Drives Inhibition of Nuclear Import,
Loss of Nuclear TDP-43, and Cell Death. Neuron
Volume 102, Issue 2, 17 April 2019, Pages 339-357.e7