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Select item 101404441.

Regional and age-dependent changes in ubiquitination in cellular and mouse models of spinocerebellar ataxia type 3

Haiyang Luo, Sokol V. Todi, Henry L. Paulson, Maria do Carmo Costa

Front Mol Neurosci. 2023; 16: 1154203. Published online 2023 Apr 14. doi: 10.3389/fnmol.2023.1154203

PMCID:: PMC10140444

Abstract Article PDF–2.5M

Select item 99753942.

Disease-associated oligodendrocyte signatures are spatiotemporally dysregulated in spinocerebellar ataxia type 3

Kristen H. Schuster, Danielle M. DiFranco, Alexandra F. Putka, Juan P. Mato, Sabrina I. Jarrah, Nicholas R. Stec, Vikram O. Sundararajan, Hayley S. McLoughlin

Front Neurosci. 2023; 17: 1118429. Published online 2023 Feb 15. doi: 10.3389/fnins.2023.1118429

PMCID:: PMC9975394

Abstract Article PDF–4.6M

Select item 94065613.

Pathogenetic Mechanisms Underlying Spinocerebellar Ataxia Type 3 Are Altered in Primary Oligodendrocyte Culture

Kristen H. Schuster, Alexandra F. Putka, Hayley S. McLoughlin

Cells. 2022 Aug; 11(16): 2615. Published online 2022 Aug 22. doi: 10.3390/cells11162615

PMCID:: PMC9406561

Abstract Article PDF–5.1M

Select item 87867554.

A Novel SCA3 Knock-in Mouse Model Mimics the Human SCA3 Disease Phenotype Including Neuropathological, Behavioral, and Transcriptional Abnormalities Especially in Oligodendrocytes

Eva Haas, Rana D. Incebacak, Thomas Hentrich, Chrisovalantou Huridou, Thorsten Schmidt, Nicolas Casadei, Yacine Maringer, Carola Bahl, Frank Zimmermann, James D. Mills, Eleonora Aronica, Olaf Riess, Julia M. Schulze-Hentrich, Jeannette Hübener-Schmid

Mol Neurobiol. 2022; 59(1): 495–522. Published online 2021 Oct 30. doi: 10.1007/s12035-021-02610-8

PMCID:: PMC8786755

Abstract Article PDF–10M

Select item 82486835.

Broad Influence of Mutant Ataxin-3 on the Proteome of the Adult Brain, Young Neurons, and Axons Reveals Central Molecular Processes and Biomarkers in SCA3/MJD Using Knock-In Mouse Model

Kalina Wiatr, Łukasz Marczak, Jean-Baptiste Pérot, Emmanuel Brouillet, Julien Flament, Maciej Figiel

Front Mol Neurosci. 2021; 14: 658339. Published online 2021 Jun 17. doi: 10.3389/fnmol.2021.658339

PMCID:: PMC8248683

Abstract Article PDF–10M

Select item 79803456.

Neurodegenerative phosphoprotein signaling landscape in models of SCA3

Anna S. Sowa, Taissia G. Popova, Tina Harmuth, Jonasz J. Weber, Priscila Pereira Sena, Jana Schmidt, Jeannette Hübener-Schmid, Thorsten Schmidt

Mol Brain. 2021; 14: 57. Published online 2021 Mar 19. doi: 10.1186/s13041-020-00723-0

PMCID:: PMC7980345

Abstract Article PDF–1.5M

Select item 61922847.

Mitochondrial Morphology, Function and Homeostasis Are Impaired by Expression of an N-terminal Calpain Cleavage Fragment of Ataxin-3

Tina Harmuth, Caroline Prell-Schicker, Jonasz J. Weber, Frank Gellerich, Claudia Funke, Stefan Drießen, Janine C. D. Magg, Guido Krebiehl, Hartwig Wolburg, Stefanie N. Hayer, Stefan Hauser, Rejko Krüger, Ludger Schöls, Olaf Riess, Jeannette Hübener-Schmid

Front Mol Neurosci. 2018; 11: 368. Published online 2018 Oct 10. doi: 10.3389/fnmol.2018.00368

PMCID:: PMC6192284

Abstract Article PDF–6.1M

Select item 61455298.

Loss of the Spinocerebellar Ataxia type 3 disease protein ATXN3 alters transcription of multiple signal transduction pathways

Li Zeng, Dapeng Zhang, Hayley S. McLoughlin, Annie J. Zalon, L. Aravind, Henry L. Paulson

PLoS One. 2018; 13(9): e0204438. Published online 2018 Sep 19. doi: 10.1371/journal.pone.0204438

PMCID:: PMC6145529

Abstract Article PDF–3.7M

Select item 55525889.

PolyQ-expanded ataxin-3 interacts with full-length ataxin-3 in a polyQ length-dependent manner

Na-Li Jia, Er-Kang Fei, Zheng Ying, Hong-Feng Wang, Guang-Hui Wang

Neurosci Bull. 2008 Aug; 24(4): 201–208. Published online 2008 Aug 2. doi: 10.1007/s12264-008-0326-9

PMCID:: PMC5552588

Abstract Article PDF–1.1M

Select item 547678610.

The Truncated C-terminal Fragment of Mutant ATXN3 Disrupts Mitochondria Dynamics in Spinocerebellar Ataxia Type 3 Models

Jung-Yu Hsu, Yu-Ling Jhang, Pei-Hsun Cheng, Yu-Fan Chang, Su-Han Mao, Han-In Yang, Chia-Wei Lin, Chuan-Mu Chen, Shang-Hsun Yang

Front Mol Neurosci. 2017; 10: 196. Published online 2017 Jun 20. doi: 10.3389/fnmol.2017.00196

PMCID:: PMC5476786

Abstract Article PDF–600K

Select item 432143811.

A knockin mouse model of spinocerebellar ataxia type 3 exhibits prominent aggregate pathology and aberrant splicing of the disease gene transcript

Biswarathan Ramani, Ginny M. Harris, Rogerio Huang, Takahiro Seki, Geoffrey G. Murphy, Maria do Carmo Costa, Svetlana Fischer, Thomas L. Saunders, Guangbin Xia, Richard C. McEachin, Henry L. Paulson

Hum Mol Genet. 2015 Mar 1; 24(5): 1211–1224. Published online 2014 Oct 15. doi: 10.1093/hmg/ddu532

Correction in:: Hum Mol Genet. 2017 Aug 15; 26(16): 3232–3233.

PMCID:: PMC4321438

Abstract Article PDF–1.1M

Select item 396613912.

The de-ubiquitinating enzyme ataxin-3 does not modulate disease progression in a knock-in mouse model of Huntington disease

Li Zeng, Sara J. Tallaksen-Greene, Bo Wang, Roger L. Albin, Henry L. Paulson

J Huntingtons Dis. Author manuscript; available in PMC 2014 Mar 26.

Published in final edited form as: J Huntingtons Dis. 2013 Jun 18; 2(1): 201–215. doi: 10.3233/JHD-130058

PMCID:: PMC3966139

Abstract Article PDF–3.0M

Select item 363392013.

Cerebellar Soluble Mutant Ataxin-3 Level Decreases during Disease Progression in Spinocerebellar Ataxia Type 3 Mice

Huu Phuc Nguyen, Jeannette Hübener, Jonasz Jeremiasz Weber, Stephan Grueninger, Olaf Riess, Andreas Weiss

PLoS One. 2013; 8(4): e62043. Published online 2013 Apr 23. doi: 10.1371/journal.pone.0062043

PMCID:: PMC3633920

Abstract Article PDF–6.5M

Select item 346868314.

Basal and stress-induced Hsp70 are modulated by ataxin-3

Christopher P. Reina, Barzin Y. Nabet, Peter D. Young, Randall N. Pittman

Cell Stress Chaperones. 2012 Nov; 17(6): 729–742. Published online 2012 Jul 10. doi: 10.1007/s12192-012-0346-2

PMCID:: PMC3468683

Abstract Article PDF–646K

Select item 282544915.

Nuclear Aggregation of Polyglutamine-expanded Ataxin-3: FRAGMENTS ESCAPE THE CYTOPLASMIC QUALITY CONTROL

Peter Breuer, Annette Haacke, Bernd O. Evert, Ullrich Wüllner

J Biol Chem. 2010 Feb 26; 285(9): 6532–6537. Published online 2010 Jan 11. doi: 10.1074/jbc.M109.036335

PMCID:: PMC2825449

Abstract Article PDF–2.5M

Select item 588617516.

Comparison of spinocerebellar ataxia type 3 mouse models identifies early gain-of-function, cell-autonomous transcriptional changes in oligodendrocytes

Biswarathan Ramani, Bharat Panwar, Lauren R. Moore, Bo Wang, Rogerio Huang, Yuanfang Guan, Henry L. Paulson

Hum Mol Genet. 2017 Sep 1; 26(17): 3362–3374. Published online 2017 Jun 13. doi: 10.1093/hmg/ddx224

PMCID:: PMC5886175

Abstract Article PDF–1.2M

Select item 462423317.

Limited Effect of Chronic Valproic Acid Treatment in a Mouse Model of Machado-Joseph Disease

Sofia Esteves, Sara Duarte-Silva, Luana Naia, Andreia Neves-Carvalho, Andreia Teixeira-Castro, Ana Cristina Rego, Anabela Silva-Fernandes, Patrícia Maciel

PLoS One. 2015; 10(10): e0141610. Published online 2015 Oct 27. doi: 10.1371/journal.pone.0141610

PMCID:: PMC4624233

Abstract Article PDF–2.4M

Select item 304482818.

Mouse Ataxin-3 Functional Knock-Out Model

Pawel M. Switonski, Agnieszka Fiszer, Katarzyna Kazmierska, Maciej Kurpisz, Wlodzimierz J. Krzyzosiak, Maciej Figiel

Neuromolecular Med. 2011; 13(1): 54–65. Published online 2010 Oct 14. doi: 10.1007/s12017-010-8137-3

PMCID:: PMC3044828

Abstract Article PDF–622K

Select item 289408219.

Genomic structure, promoter activity, and developmental expression of the mouse homologue of the Machado–Joseph disease (MJD) gene

Maria do Carmo Costa, Joana Gomes-da-Silva, Carlos J. Miranda, Jorge Sequeiros, Manuela M. Santos, Patrícia Maciel

Genomics. Author manuscript; available in PMC 2010 Jun 29.

Published in final edited form as: Genomics. 2004 Aug; 84(2): 361–373. doi: 10.1016/j.ygeno.2004.02.012

PMCID:: PMC2894082

Abstract Article PDF–2.7M

Select item 925953320.

KPNB1 modulates the Machado–Joseph disease protein ataxin-3 through activation of the mitochondrial protease CLPP

Mahkameh Abeditashi, Jonasz Jeremiasz Weber, Priscila Pereira Sena, Ana Velic, Maria Kalimeri, Rana Dilara Incebacak Eltemur, Jana Schmidt, Jeannette Hübener-Schmid, Stefan Hauser, Boris Macek, Olaf Riess, Thorsten Schmidt

Cell Mol Life Sci. 2022 Aug; 79(8): 401. Published online 2022 Jul 6. doi: 10.1007/s00018-022-04372-5