Excitation-induced ataxin-3 aggregation in neurons from patients with Machado–Joseph disease

被引：0
作者：
Philipp Koch
Peter Breuer
Michael Peitz
Johannes Jungverdorben
Jaideep Kesavan
Daniel Poppe
Jonas Doerr
Julia Ladewig
Jerome Mertens
Thomas Tüting
Per Hoffmann
Thomas Klockgether
Bernd O. Evert
Ullrich Wüllner
Oliver Brüstle
机构：
[1] Institute of Reconstructive Neurobiology,Department of Neurology
[2] Life and Brain Center,Department of Dermatology
[3] University of Bonn and Hertie Foundation,Institute of Human Genetics and Department of Genomics
[4] University of Bonn Medical Center,undefined
[5] 53105 Bonn,undefined
[6] Germany,undefined
[7] University of Bonn Medical Center,undefined
[8] 53127 Bonn,undefined
[9] Germany,undefined
[10] Life and Brain Center,undefined
[11] University of Bonn,undefined
[12] 53127 Bonn,undefined
[13] Germany,undefined
[14] DZNE,undefined
[15] German Center for Neurodegenerative Diseases,undefined
[16] 53175 Bonn,undefined
[17] Germany,undefined
来源：
Nature | 2011年 / 480卷
关键词：
D O I：
暂无
中图分类号：
学科分类号：
摘要：
Human neurons derived from induced pluripotent stem cells permit the study of aberrant protein processing and aggregation in Machado–Joseph disease.
引用
收藏
页码：543 / 546
页数：3
相关论文
共 50 条
[41]   Calpastatin-mediated inhibition of calpains in the mouse brain prevents mutant ataxin 3 proteolysis, nuclear localization and aggregation, relieving Machado-Joseph disease [J].
Simoes, Ana T. ;
Goncalves, Nelio ;
Koeppen, Arnulf ;
Deglon, Nicole ;
Kuegler, Sebastian ;
Duarte, Carlos Bandeira ;
de Almeida, Luis Pereira .
BRAIN, 2012, 135 :2428-2439
[42]   Splice Isoforms of the Polyglutamine Disease Protein Ataxin-3 Exhibit Similar Enzymatic yet Different Aggregation Properties [J].
Harris, Ginny Marie ;
Dodelzon, Katerina ;
Gong, Lijie ;
Gonzalez-Alegre, Pedro ;
Paulson, Henry L. .
PLOS ONE, 2010, 5 (10)
[43]   CDK5 protects from caspase-induced Ataxin-3 cleavage and neurodegeneration [J].
Liman, Jan ;
Deeg, Sebastian ;
Voigt, Aaron ;
Vossfeldt, Hannes ;
Dohm, Christoph P. ;
Karch, Andre ;
Weishaupt, Jochen ;
Schulz, Joerg B. ;
Baehr, Mathias ;
Kermer, Pawel .
JOURNAL OF NEUROCHEMISTRY, 2014, 129 (06) :1013-1023
[44]   Survival estimates for patients with Machado-Joseph disease (SCA3) [J].
Kieling, C. ;
Prestes, P. R. ;
Saraiva-Pereira, M. L. ;
Jardim, Laura Bannach .
CLINICAL GENETICS, 2007, 72 (06) :543-545
[45]   Inhibition of Transglutaminase Exacerbates Polyglutamine-Induced Neurotoxicity by Increasing the Aggregation of Mutant Ataxin-3 in an SCA3 Drosophila Model [J].
Lin, Yunting ;
He, Hua ;
Luo, Yingying ;
Zhu, Ting ;
Duan, Ranhui .
NEUROTOXICITY RESEARCH, 2015, 27 (03) :259-267
[46]   Inhibition of Transglutaminase Exacerbates Polyglutamine-Induced Neurotoxicity by Increasing the Aggregation of Mutant Ataxin-3 in an SCA3 Drosophila Model [J].
Yunting Lin ;
Hua He ;
Yingying Luo ;
Ting Zhu ;
Ranhui Duan .
Neurotoxicity Research, 2015, 27 :259-267
[47]   Toxicity and aggregation of the polyglutamine disease protein, ataxin-3 is regulated by its binding to VCP/p97 in Drosophila melanogaster [J].
Ristic, Gorica ;
Sutton, Joanna R. ;
Libohova, Kozeta ;
Todi, Sokol V. .
NEUROBIOLOGY OF DISEASE, 2018, 116 :78-92
[48]   Depressive symptoms in Machado-Joseph disease (SCA3) patients and their relatives [J].
Cecchin, C. R. ;
Pires, A. P. ;
Rieder, C. R. ;
Monte, T. L. ;
Silveira, I. ;
Carvalho, T. ;
Saraiva-Pereira, M. L. ;
Sequeiros, J. ;
Jardim, L. B. .
COMMUNITY GENETICS, 2007, 10 (01) :19-26
[49]   Eye Movement Abnormalities in patients with Spinocerebellar ataxia 3/Machado-Joseph disease [J].
Ghasia, Fatema Firoz ;
Wilmot, George ;
Ahmed, Anwar ;
Shaikh, Aasef .
INVESTIGATIVE OPHTHALMOLOGY & VISUAL SCIENCE, 2015, 56 (07)
[50]   Citalopram Reduces Aggregation of ATXN3 in a YAC Transgenic Mouse Model of Machado-Joseph Disease [J].
Ashraf, Naila S. ;
Duarte-Silva, Sara ;
Shaw, Emily D. ;
Maciel, Patricia ;
Paulson, Henry L. ;
Teixeira-Castro, Andreia ;
Costa, Maria do Carmo .
MOLECULAR NEUROBIOLOGY, 2019, 56 (05) :3690-3701
12345