Results 171 to 180 of about 13,262,735 (197)

Temperature profoundly affects ataxin-3 fibrillogenesis

Biochimie, 2012
Ataxin-3 (AT3) triggers spinocerebellar ataxia type 3 when it carries a polyglutamine stretch expanded beyond a critical threshold. By Fourier transform infrared spectroscopy and atomic force microscopy we previously showed that a normal (AT3Q24) and an expanded (AT3Q55) variant were capable of evolving into oligomers and protofibrils at 37 °C, whereas
A. Apicella, A. Natalello, A. M. Frana, A. Baserga, CASARI, CARLO SPARTACO, BOTTANI, CARLO ENRICO, S. M. Doglia, P. Tortora, M. E. Regonesi   +8 more
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Structural and functional analysis of ataxin‐2 and ataxin‐3

European Journal of Biochemistry, 2004
Spinocerebellar ataxia types 2 (SCA2) and 3 (SCA3) are autosomal‐dominantly inherited, neurodegenerative diseases caused by CAG repeat expansions in the coding regions of the genes encoding ataxin‐2 and ataxin‐3, respectively. To provide a rationale for further functional experiments, we explored the protein architectures of ataxin‐2 and ataxin‐3 ...
Albrecht, M., Golatta, M., Wüllner, U., Lengauer, T.   +3 more
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Aggregation of the polyglutamine protein ataxin-3

2021
This thesis was scanned from the print manuscript for digital preservation and is copyright the author. Researchers can access this thesis by asking their local university, institution or public library to make a request on their behalf. Monash staff and postgraduate students can use the link in the References field.
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Dynamic expression of Hsp27 in the presence of mutant ataxin-3

Biochemical and Biophysical Research Communications, 2005
Machado-Joseph disease (MJD)/spinocerebellar ataxia type 3 (SCA3) is an autosomal dominant spinocerebellar degeneration characterized by a wide range of clinical manifestations. The molecular mechanisms underlying the selective neuronal death typical of MJD/SCA3 are unknown.
Wei-Hsiu, Chang, Cemal K, Cemal, Yung-Hsiang, Hsu, Cheng-Liang, Kuo, Nobuyuki, Nukina, Ming-Hong, Chang, Hsiao-Tang, Hu, Chuan, Li, Mingli, Hsieh   +8 more
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Mechanisms of ataxin-3 misfolding and fibril formation

2021
This thesis was scanned from the print manuscript for digital preservation and is copyright the author. Researchers can access this thesis by asking their local university, institution or public library to make a request on their behalf. Monash staff and postgraduate students can use the link in the References field.
openaire   +1 more source

Trehalose prevents the formation of aggregates of mutant ataxin-3 and reduces soluble ataxin-3 protein levels in an SCA3 cell model

Neuroscience
Spinocerebellar ataxia type 3 (SCA3) is a neurodegenerative disorder caused by mutant ataxin-3 with an abnormally expanded polyQ tract and is the most common dominantly inherited ataxia worldwide. There are no suitable therapeutic options for this disease. Autophagy, a defense mechanism against the toxic effects of aggregation-prone misfolded proteins,
Zijian, Wang, Min, Wang, Yuhang, Huang, Zhiwei, Ma, Wenjing, Gao, Tian, Zhang, Jiexin, Deng, Xiaoxia, Cheng, Yingxun, Liu, Bo, Wang, Ying, Qi, Min, Yang, Fengqin, He   +12 more
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Hydrostatic Pressure Effects on the Stability of Ataxin-3

2003
Protein misfolding and formation of amyloids are the major event in the development of neurodegenerative diseases but the mechanism of this biological phenomenon remains to be elucidated. Here, we report the high pressure denaturation of the spinocerebellar ataxia type 3 protein Ataxin-3 from man and mouse using fluorescence spectroscopy.
Stéphane Marchal, Erlet Shehi, Paola Fusi, Paolo Tortora, Reinhard Lange   +4 more
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Evolution and functional relevance of Ataxin-3 paralogues

2013
Ataxin-3 gene (ATXN3; 14q32.1) encodes a ubiquitously expressed protein involved in the ubiquitin-proteasome pathway and in transcription repression. Much attention has been given to ATXN3 since the identification of an expanded (CAG)n tract in its coding region, responsible for the most common dominant ataxia worldwide, SCA3 or Machado-Joseph disease (
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Ataxin-3

2013
Sokol V. Todi, Annalisa Pastore, Henry L. Paulson   +2 more
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[Polyglutamine-expanded ataxin-3 is degraded by autophagy].

Zhonghua yi xue yi chuan xue za zhi = Zhonghua yixue yichuanxue zazhi = Chinese journal of medical genetics, 2010
To investigate the role of autophagy on the pathogenesis of spinocerebellar ataxia 3/Machado-Joseph disease (SCA3/MJD).HEK293 cells expressing polyglutamine-expanded ataxin-3 were used as cell model for SCA3/MJD. The level of polyglutamine-expanded ataxin-3 was detected after cells were treated with different inhibitors or inducer of autophagy ...
Han, Xiao, Jianguang, Tang, Zhiping, Hu, Jieqiong, Tan, Beisha, Tang, Zheng, Jiang   +5 more
openaire   +1 more source