The Ins and Outs of a Polyglutamine Neurodegenerative Disease: Spinocerebellar Ataxia Type 1 (SCA1)

by Harry T. Orr
Published 2000-06-01

Reduction of Purkinje Cell Pathology in SCA1 Transgenic Mice by p53 Deletion

by Mona D. Shahbazian, Harry T. Orr, Huda Y. Zoghbi
Published 2001-12-01

Polarization-sensitive optical coherence tomography reveals gray matter and white matter atrophy in SCA1 mouse models

by Chao J. Liu, Orion Rainwater, H. Brent Clark, Harry T. Orr, Taner Akkin
Published 2018-08-01

Antisense RNA Sequences Modulating the Ataxin-1 Message: Molecular Model of Gene Therapy for Spinocerebellar Ataxia Type 1, a Dominant-Acting Unstable Trinucleotide Repeat Disease

by Youxin Gao, Tao Zu, Walter C. Low, Harry T. Orr, R. Scott Mcivor Ph.D.
Published 2008-07-01

Partial loss of ataxin-1 function contributes to transcriptional dysregulation in spinocerebellar ataxia type 1 pathogenesis.

by Juan Crespo-Barreto, John D Fryer, Chad A Shaw, Harry T Orr, Huda Y Zoghbi
Published 2010-07-01

Assessing recovery from neurodegeneration in spinocerebellar ataxia 1: Comparison of in vivo magnetic resonance spectroscopy with motor testing, gene expression and histology

by Gülin Öz, Emily Kittelson, Döne Demirgöz, Orion Rainwater, Lynn E. Eberly, Harry T. Orr, H. Brent Clark
Published 2015-02-01

Motor neuron degeneration correlates with respiratory dysfunction in SCA1

by James P. Orengo, Meike E. van der Heijden, Shuang Hao, Jianrong Tang, Harry T. Orr, Huda Y. Zoghbi
Published 2018-02-01

Lithium therapy improves neurological function and hippocampal dendritic arborization in a spinocerebellar ataxia type 1 mouse model.

by Kei Watase, Jennifer R Gatchel, Yaling Sun, Effat Emamian, Richard Atkinson, Ronald Richman, Hidehiro Mizusawa, Harry T Orr, Chad Shaw, Huda Y Zoghbi
Published 2007-05-01

Treadmill training increases the motor activity and neuron survival of the cerebellum in a mouse model of spinocerebellar ataxia type 1

by Chieh‐Sen Chuang, Jui‐Chih Chang, Bing‐Wen Soong, Sheng‐Fei Chuang, Ta‐Tsung Lin, Wen‐Ling Cheng, Harry T. Orr, Chin‐San Liu
Published 2019-11-01

The ataxin-1 interactome reveals direct connection with multiple disrupted nuclear transport pathways

by Sunyuan Zhang, Nicholas A. Williamson, Lisa Duvick, Alexander Lee, Harry T. Orr, Austin Korlin-Downs, Praseuth Yang, Yee-Foong Mok, David A. Jans, Marie A. Bogoyevitch
Published 2020-07-01

Modulation of ATXN1 S776 phosphorylation reveals the importance of allele-specific targeting in SCA1

by Larissa Nitschke, Stephanie L. Coffin, Eder Xhako, Dany B. El-Najjar, James P. Orengo, Elizabeth Alcala, Yanwan Dai, Ying-Wooi Wan, Zhandong Liu, Harry T. Orr, Huda Y. Zoghbi
Published 2021-02-01

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

by Cristian A Lasagna-Reeves, Maxime WC Rousseaux, Marcos J Guerrero-Munoz, Luis Vilanova-Velez, Jeehye Park, Lauren See, Paymaan Jafar-Nejad, Ronald Richman, Harry T Orr, Rakez Kayed, Huda Y Zoghbi
Published 2015-12-01

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

by Cristian A Lasagna-Reeves, Maxime WC Rousseaux, Marcos J Guerrero-Muñoz, Jeehye Park, Paymaan Jafar-Nejad, Ronald Richman, Nan Lu, Urmi Sengupta, Alexandra Litvinchuk, Harry T Orr, Rakez Kayed, Huda Y Zoghbi
Published 2015-05-01

Antisense Oligonucleotide Therapeutic Approach for Suppression of Ataxin-1 Expression: A Safety Assessment

by Brennon O’Callaghan, Bente Hofstra, Hillary P. Handler, Holly B. Kordasiewicz, Tracy Cole, Lisa Duvick, Jillian Friedrich, Orion Rainwater, Praseuth Yang, Michael Benneyworth, Tessa Nichols-Meade, Wesley Heal, Rachel Ter Haar, Christine Henzler, Harry T. Orr
Published 2020-09-01

Reduction of protein kinase A-mediated phosphorylation of ATXN1-S776 in Purkinje cells delays onset of Ataxia in a SCA1 mouse model

by Judit M. Pérez Ortiz, Nissa Mollema, Nicholas Toker, Carolyn J. Adamski, Brennon O'Callaghan, Lisa Duvick, Jillian Friedrich, Michael A. Walters, Jessica Strasser, Jon E. Hawkinson, Huda Y. Zoghbi, Christine Henzler, Harry T. Orr, Sarita Lagalwar
Published 2018-08-01