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Select item 106889821.

CRISPR screening identifies the deubiquitylase ATXN3 as a PD-L1–positive regulator for tumor immune evasion

Shengnan Wang, Radhika Iyer, Xiaohua Han, Juncheng Wei, Na Li, Yang Cheng, Yuanzhang Zhou, Qiong Gao, Lingqiang Zhang, Ming Yan, Zhaolin Sun, Deyu Fang

J Clin Invest. 2023 Dec 1; 133(23): e167728. Published online 2023 Dec 1. doi: 10.1172/JCI167728

PMCID:: PMC10688982

Article PDF–3.6M

Select item 102879152.

ATXN3 controls DNA replication and transcription by regulating chromatin structure

Esperanza Hernández‐Carralero, Elisa Cabrera, Gara Rodríguez-Torres, Yeray Hernández-Reyes, Abhay N Singh, Cristina Santa-María, José Miguel Fernández-Justel, Roel C Janssens, Jurgen A Marteijn, Bernd O Evert, Niels Mailand, María Gómez, Kristijan Ramadan, Veronique A J Smits, Raimundo Freire

Nucleic Acids Res. 2023 Jun 23; 51(11): 5396–5413. Published online 2023 Mar 27. doi: 10.1093/nar/gkad212

PMCID:: PMC10287915

Article PDF–2.1M

Select item 101124643.

Predominant motor neuron involvement as a manifestation of pathogenic (full range) ATXN3 mutations

Jarosław Dulski, Alexandra Estela Soto Piña, Rana Hanna Al-Shaikh, Leonard Petrucelli, Zbigniew K. Wszolek

Neurol Sci. Author manuscript; available in PMC 2024 Jan 1.

Published in final edited form as: Neurol Sci. 2023 Jan; 44(1): 389–392. Published online 2022 Sep 23. doi: 10.1007/s10072-022-06410-3

PMCID:: PMC10112464

Article PDF–665K

Select item 92222034.

A Robust Assay to Monitor Ataxin-3 Amyloid Fibril Assembly

Francisco Figueiredo, Mónica Lopes-Marques, Bruno Almeida, Nena Matscheko, Pedro M. Martins, Alexandra Silva, Sandra Macedo-Ribeiro

Cells. 2022 Jun; 11(12): 1969. Published online 2022 Jun 19. doi: 10.3390/cells11121969

PMCID:: PMC9222203

Article PDF–6.5M

Select item 90863955.

Ataxin-3, The Spinocerebellar Ataxia Type 3 Neurodegenerative Disorder Protein, Affects Mast Cell Functions

Anna S. Sowa, Eva Haas, Jeannette Hübener-Schmid, Axel Lorentz

Front Immunol. 2022; 13: 870966. Published online 2022 Apr 26. doi: 10.3389/fimmu.2022.870966

PMCID:: PMC9086395

Article PDF–2.4M

Select item 88361696.

Anti-Excitotoxic Effects of N-Butylidenephthalide Revealed by Chemically Insulted Purkinje Progenitor Cells Derived from SCA3 iPSCs

Hsin-Han Yang, I-Tsang Chiang, Jen-Wei Liu, Jeanne Hsieh, Jui-Hao Lee, Huai-En Lu, Hwa-Sung Tso, Yu-Chen Deng, Jo-Chi Kao, Jhen-Rong Wu, Horng-Jyh Harn, Tzyy-Wen Chiou

Int J Mol Sci. 2022 Feb; 23(3): 1391. Published online 2022 Jan 26. doi: 10.3390/ijms23031391

PMCID:: PMC8836169

Article PDF–2.5M

Select item 86930827.

Degron capability of the hydrophobic C-terminus of the polyglutamine disease protein, ataxin-3

Jessica R. Blount, Sean L. Johnson, Kozeta Libohova, Sokol V. Todi, Wei-Ling Tsou

J Neurosci Res. Author manuscript; available in PMC 2021 Dec 22.

Published in final edited form as: J Neurosci Res. 2020 Oct; 98(10): 2096–2108. Published online 2020 Jul 9. doi: 10.1002/jnr.24684

PMCID:: PMC8693082

Article PDF–1.8M

Select item 79308868.

Antisense oligonucleotide therapy targeted against ATXN3 improves potassium channel-mediated Purkinje neuron dysfunction in spinocerebellar ataxia type 3

David D. Bushart, Annie J. Zalon, Hongjiu Zhang, Logan M. Morrison, Yuanfang Guan, Henry L. Paulson, Vikram G. Shakkottai, Hayley S. McLoughlin

Cerebellum. Author manuscript; available in PMC 2022 Feb 1.

Published in final edited form as: Cerebellum. 2021 Feb; 20(1): 41–53. doi: 10.1007/s12311-020-01179-7

PMCID:: PMC7930886

Article PDF–934K

Select item 79061509.

Polyglutamine-expanded ataxin3 alter specific gene expressions through changing DNA methylation status in SCA3/MJD

Dongxue Ding, Chunrong Wang, Zhao Chen, Kun Xia, Beisha Tang, Rong Qiu, Hong Jiang

Aging (Albany NY) 2021 Feb 15; 13(3): 3680–3698. Published online 2020 Dec 19. doi: 10.18632/aging.202331

PMCID:: PMC7906150

Article PDF–1.3M

Select item 783573210.

Pathological ATX3 Expression Induces Cell Perturbations in E. coli as Revealed by Biochemical and Biophysical Investigations

Diletta Ami, Barbara Sciandrone, Paolo Mereghetti, Jacopo Falvo, Tiziano Catelani, Cristina Visentin, Paolo Tortora, Salvador Ventura, Antonino Natalello, Maria Elena Regonesi

Int J Mol Sci. 2021 Jan; 22(2): 943. Published online 2021 Jan 19. doi: 10.3390/ijms22020943

PMCID:: PMC7835732

Article PDF–5.3M

Select item 750710011.

Clinical features and genetic characteristics of homozygous spinocerebellar ataxia type 3

Quan-Fu Li, Hao‐Ling Cheng, Lu Yang, Yin Ma, Jing‐Jing Zhao, Yi Dong, Zhi‐Ying Wu

Mol Genet Genomic Med. 2020 Sep; 8(9): e1314. Published online 2020 Jul 9. doi: 10.1002/mgg3.1314

PMCID:: PMC7507100

Article PDF–860K

Select item 687153512.

Yemenite-Jewish families with Machado–Joseph disease (MJD/SCA3) share a recent common ancestor

Reuven Sharony, Sandra Martins, Inês P. D. Costa, Roy Zaltzman, António Amorim, Jorge Sequeiros, Carlos R. Gordon

Eur J Hum Genet. 2019 Nov; 27(11): 1731–1737. Published online 2019 Jun 12. doi: 10.1038/s41431-019-0449-7

PMCID:: PMC6871535

Article PDF–610K

Select item 637344113.

Abnormal eyeblink conditioning is an early marker of cerebellar dysfunction in preclinical SCA3 mutation carriers

J. van Gaalen, R. P. P. W. M. Maas, E. F. Ippel, M. W. Elting, K. Y. van Spaendonck-Zwarts, S. Vermeer, C. Verschuuren-Bemelmans, D. Timmann, Bart P. van de Warrenburg

Exp Brain Res. 2019; 237(2): 427–433. Published online 2018 Nov 14. doi: 10.1007/s00221-018-5424-y

PMCID:: PMC6373441

Article PDF–745K

Select item 509948714.

Autophagy Promoted the Degradation of Mutant ATXN3 in Neurally Differentiated Spinocerebellar Ataxia-3 Human Induced Pluripotent Stem Cells

Zhanhui Ou, Min Luo, Xiaohua Niu, Yuchang Chen, Yingjun Xie, Wenyin He, Bing Song, Yexing Xian, Di Fan, Shuming OuYang, Xiaofang Sun

Biomed Res Int. 2016; 2016: 6701793. Published online 2016 Oct 25. doi: 10.1155/2016/6701793

PMCID:: PMC5099487

Article PDF–3.8M

Select item 452516115.

Infrared nanospectroscopy characterization of oligomeric and fibrillar aggregates during amyloid formation

F. S. Ruggeri, G. Longo, S. Faggiano, E. Lipiec, A. Pastore, G. Dietler

Nat Commun. 2015; 6: 7831. Published online 2015 Jul 28. doi: 10.1038/ncomms8831

PMCID:: PMC4525161

Article PDF–3.7M

Select item 682619216.

The p97–Ataxin 3 complex regulates homeostasis of the DNA damage response E3 ubiquitin ligase RNF8

Abhay Narayan Singh, Judith Oehler, Ignacio Torrecilla, Susan Kilgas, Shudong Li, Bruno Vaz, Claire Guérillon, John Fielden, Esperanza Hernandez‐Carralero, Elisa Cabrera, Iain DC Tullis, Mayura Meerang, Paul R Barber, Raimundo Freire, Jason Parsons, Borivoj Vojnovic, Anne E Kiltie, Niels Mailand, Kristijan Ramadan

EMBO J. 2019 Nov 4; 38(21): e102361. Published online 2019 Oct 15. doi: 10.15252/embj.2019102361

PMCID:: PMC6826192

Article PDF–7.3M

Select item 673017917.

A Mutant Ataxin-3 Putative-Cleavage Fragment in Brains of Machado-Joseph Disease Patients and Transgenic Mice Is Cytotoxic above a Critical Concentration

Daniel Goti, Scott M. Katzen, Jesse Mez, Noam Kurtis, Jennifer Kiluk, Lea Ben-Haïem, Nancy A. Jenkins, Neal G. Copeland, Akira Kakizuka, Alan H. Sharp, Christopher A. Ross, Peter R. Mouton, Veronica Colomer

J Neurosci. 2004 Nov 10; 24(45): 10266–10279. doi: 10.1523/JNEUROSCI.2734-04.2004