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Structural modeling of ataxin‐3 reveals distant homology to adaptins
Proteins: Structure, Function, and Bioinformatics, 2002AbstractSpinocerebellar ataxia type 3 (SCA3) is a polyglutamine disorder caused by a CAG repeat expansion in the coding region of a gene encoding ataxin‐3, a protein of yet unknown function. Based on a comprehensive computational analysis, we propose a structural model and structure‐based functions for ataxin‐3.
Albrecht, Mario +5 more
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Mechanisms of ataxin-3 misfolding and fibril formation
2021This 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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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 +12 more
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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 +12 more
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Hydrostatic Pressure Effects on the Stability of Ataxin-3
2003Protein 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 +4 more
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Evolution and functional relevance of Ataxin-3 paralogues
2013Ataxin-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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Biophysical Characterisation of the Josephin Domain of Ataxin-3
2017The misfolding and subsequent aggregation of proteins underpins a large number of conditions called conformational diseases. Many of these diseases are caused by genetic mutations in protein coding regions. One such family is the polyglutamine diseases, characterised by the expansion of a naturally occurring glutamine repeat beyond a specific threshold
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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, 2010To 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 +5 more
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