Regional rescue of spinocerebellar ataxia type 1 phenotypes by 14-3-3ε haploinsufficiency in mice underscores complex pathogenicity in neurodegeneration

Por Jafar-Nejad, Paymaan, Ward, Christopher S., Richman, Ronald, Orr, Harry T., Zoghbi, Huda Y.
Publicado em 2011

Fragile X-like behaviors and abnormal cortical dendritic spines in Cytoplasmic FMR1-interacting protein 2-mutant mice

Por Han, Kihoon, Chen, Hogmei, Gennarino, Vincenzo A., Richman, Ronald, Lu, Hui-Chen, Zoghbi, Huda Y.
Publicado no Hum Mol Genet (2015)

Opposing effects of polyglutamine expansion on native protein complexes contribute to SCA1

Por Lim, Janghoo, Crespo-Barreto, Juan, Jafar-Nejad, Paymaan, Bowman, Aaron B., Richman, Ronald, Hill, David E., Orr, Harry T., Zoghbi, Huda Y.
Publicado em 2008

Lithium Therapy Improves Neurological Function and Hippocampal Dendritic Arborization in a Spinocerebellar Ataxia Type 1 Mouse Model

Por Watase, Kei, Gatchel, Jennifer R, Sun, Yaling, Emamian, Effat, Atkinson, Richard, Richman, Ronald, Mizusawa, Hidehiro, Orr, Harry T, Shaw, Chad, Zoghbi, Huda Y
Publicado em 2007

Polyglutamine Disease Toxicity Is Regulated by Nemo-like Kinase in Spinocerebellar Ataxia Type 1

Por Ju, Hyoungseok, Kokubu, Hiroshi, Todd, Tiffany W., Kahle, Juliette J., Kim, Soeun, Richman, Ronald, Chirala, Karthik, Orr, Harry T., Zoghbi, Huda Y., Lim, Janghoo
Publicado em 2013

Ataxin-1 oligomers induce local spread of pathology and decreasing them by passive immunization slows Spinocerebellar ataxia type 1 phenotypes

Por Lasagna-Reeves, Cristian A, Rousseaux, Maxime WC, Guerrero-Munoz, Marcos J, Vilanova-Velez, Luis, Park, Jeehye, See, Lauren, Jafar-Nejad, Paymaan, Richman, Ronald, Orr, Harry T, Kayed, Rakez, Zoghbi, Huda Y
Publicado no eLife (2015)

A native interactor scaffolds and stabilizes toxic ATAXIN-1 oligomers in SCA1

Por Lasagna-Reeves, Cristian A, Rousseaux, Maxime WC, Guerrero-Muñoz, Marcos J, Park, Jeehye, Jafar-Nejad, Paymaan, Richman, Ronald, Lu, Nan, Sengupta, Urmi, Litvinchuk, Alexandra, Orr, Harry T, Kayed, Rakez, Zoghbi, Huda Y
Publicado no eLife (2015)

Genetic screens reveal RAS/MAPK/MSK1 modulate ataxin 1 protein levels and toxicity in SCA1

Por Park, Jeehye, Al-Ramahi, Ismael, Tan, Qiumin, Mollema, Nissa, Diaz-Garcia, Javier R., Gallego-Flores, Tatiana, Lu, Hsiang-Chih, Lagalwar, Sarita, Duvick, Lisa, Kang, Hyojin, Lee, Yoontae, Jafar-Nejad, Paymaan, Sayegh, Layal S., Richman, Ronald, Liu, Xiuyun, Gao, Yan, Shaw, Chad A., Arthur, J. Simon C., Orr, Harry T., Westbrook, Thomas F., Botas, Juan, Zoghbi, Huda Y.
Publicado em 2013

Disruption of the ATXN1-CIC complex causes a spectrum of neurobehavioral phenotypes in mice and humans

Por Lu, Hsiang-Chih, Tan, Qiumin, Rousseaux, Maxime WC, Wang, Wei, Kim, Ji-Yoen, Richman, Ronald, Wan, Ying-Wooi, Yeh, Szu-Ying, Patel, Jay M, Liu, Xiuyun, Lin, Tao, Lee, Yoontae, Fryer, John D, Han, Jing, Chahrour, Maria, Finnell, Richard H, Lei, Yunping, Zurita-Jimenez, Maria E, Ahimaz, Priyanka, Anyane-Yeboa, Kwame, Van Maldergem, Lionel, Lehalle, Daphne, Jean-Marcais, Nolwenn, Mosca-Boidron, Anne-Laure, Thevenon, Julien, Cousin, Margot A, Bro, Della E, Lanpher, Brendan C, Klee, Eric W, Alexander, Nora, Bainbridge, Matthew N, Orr, Harry T, Sillitoe, Roy V, Ljungberg, M. Cecilia, Liu, Zhandong, Schaaf, Christian P, Zoghbi, Huda Y
Publicado no Nat Genet (2017)