Gendron, Tania F
Mayo Clinic
Department of Neuroscience, Mayo Clinic, Jacksonville, FL 32224, USA.
Citations 13
Toward allele-specific targeting therapy and pharmacodynamic marker for spinocerebellar ataxia type 3
Prudencio, Mercedes , García‐Moreno, Héctor , Jansen‐West, Karen , Al‐Shaikh, Rana Hanna , Gendron, Tania F. , Spiegel, Matthew R. , Carlomagno, Yari , Daughrity, Lillian M. , Song, Yuping , Dunmore, Judith A. , Byron, Natalie, Oskarsson, Björn , Nicholson, Katharine, Staff, Nathan P. , Gorcenco, Sorina , Puschmann, Andreas , Lemos, João , Januário, Cristina , LeDoux, Mark S. , Polke, James, Labrum, Robin, McLoughlin, Hayley S. , Paulson, Henry L. , Onodera, Osamu , Tada, Mari , Kakita, Akiyoshi , Fryer, John Denis , Karremo, Christin, Gomes, Inês , Caviness, John N., Pittelkow, Mark R. , Aasly, Jan , Pfeiffer, Ronald F. , Veerappan, Venka , Eggenberger, Eric , Huang, Josephine F. , Uitti, Ryan J. , Wierenga, Klaas J. , Collazo, I. Vanessa Marin , Tipton, Philip W. , Gerpen, Jay A. van, Blitterswijk, Marka van , Bu, Guojun , Petrucelli, Leonard , Jansen-West, Karen R , Carlomagno, Yari , Daughrity, Lillian M , Dunmore, Judith A , Oskarsson, Björn , Staff, Nathan P , Gorcenco, Sorina , Puschmann, Andreas , Lemos, João , Januário, Cristina , Shakkottai, Vikram , McLoughlin, Hayley S , Paulson, Henry L , Konno, Takuya , Onodera, Osamu , Ikeuchi, T , Tada, Mari , Gomes, Inês , Pittelkow, Mark R , Aasly, Jan , Eggenberger, Eric R, Freeman, William D , Huang, Josephine F , Marin Collazo, Iris V , Tipton, Philip W , van Blitterswijk, Marka , Bu, Guojun , Wszolek, Z.K. , Giunti, Paola , Petrucelli, Leonard , Prudencio, Mercedes , Gendron, Tania F , Hanna Al-Shaikh, Rana , Heckman, Michael G. , Friedman, Joseph H. , Konno, Takuya , Ikeuchi, Takeshi , Freeman, William D. , Wszołek, Zbigniew K.
Science Translational Medicine | October 21, 2020 | Volume: 12 | Issue: 566 | Pages: No page available
PolyQ ATXN3 is a pharmacodynamic marker for SCA3, and a SNP associated with the expanded allele may serve to specifically target mutant ATXN3 .
C9orf72 poly(GR) aggregation induces TDP-43 proteinopathy
Cook, Casey , Wu, Yanwei , Odeh, Hana M. , Gendron, Tania F. , Jansen‐West, Karen , Rosso, Giulia del, Yue, Mei, Jiang, Peizhou , Gomes, Edward, Tong, Jimei, Daughrity, Lillian M. , Avendano, Nicole M., Castanedes‐Casey, Monica , Shao, Wei , Oskarsson, Björn , Tomassy, Giulio Srubek, McCampbell, Alexander , Rigo, Frank , Zhang, Yong‐Jie , Petrucelli, Leonard , Cook, Casey N , Del Rosso, Giulia, Dickson, Dennis W , Shorter, James , Zhang, Yong-Jie , Petrucelli, Leonard , Oskarsson, Björn , Gendron, Tania F , Shao, Wei , Dickson, Dennis W. , Shorter, James
Science Translational Medicine | September 2, 2020 | Volume: 12 | Issue: 559 | Pages: No page available
G 4 C 2 repeat–derived poly(GR) plays a key role in mediating TDP-43 mislocalization and aggregation in vitro and in vivo.
Heterochromatin anomalies and double-stranded RNA accumulation underlie C9orf72 poly(PR) toxicity
Zhang, Yong‐Jie , Guo, Lin , Gonzales, Patrick , Gendron, Tania F. , Wu, Yanwei , Jansen‐West, Karen , O’Raw, Aliesha D. , Pickles, Sarah , Prudencio, Mercedes , Carlomagno, Yari , Gachechiladze, Mariam A. , Tian, Ruilin , Chew, Jeannie , DeTure, Michael , Lin, Wen-Lang, Tong, Jimei, Daughrity, Lillian M. , Yue, Mei, Song, Yuping , Andersen, Jonathan W. , Castanedes‐Casey, Monica , Kurti, Aishe, Antognetti, Giovanna, McCampbell, Alexander , Rademakers, Rosa , Oskarsson, Björn , Kampmann, Martin , Ward, Michael E. , Fryer, John Denis , Link, Christopher D. , Shorter, James , Petrucelli, Leonard , Gonzales, Patrick K , Pickles, Sarah R , Chew, Jeannie , DeTure, Michael , Daughrity, Lillian M , Castanedes-Casey, Monica , McCampbell, Alexander , Rademakers, Rosa , Oskarsson, Björn , Dickson, Dennis W , Kampmann, Martin , Ward, Michael E , Shorter, James , Petrucelli, Leonard , Zhang, Yong-Jie , Prudencio, Mercedes , O'Raw, Aliesha , Gendron, Tania F , Guo, Lin , Ludwig, Connor , Datta, Abhishek, Dickson, Dennis W.
Science | February 15, 2019 | Volume: 363 | Issue: 6428 | Pages: No page available
How dipeptide repeats cause pathology A repeat expansion in the chromosome 9 open reading frame 72 ( C9orf72 ) gene is the most common known cause of two neurodegenerative diseases: frontotemporal dementia and amyotrophic lateral sclerosis. This expansion leads to the abnormal production of proteins of repeating dipeptides, but their contribution to disease pathogenesis remains unclear. Zhang et al. engineered a mouse model to study the consequences of one of these dipeptides—prolinearginine dipeptide repeat protein, poly(PR)—in the brain. They found that poly(PR) caused neuron loss as well as motor and memory impairments. These detrimental effects resulted from poly(PR)-induced perturbation of heterochromatin function, a tightly packed form of DNA that represses gene expression. Science , this issue p. eaav2606