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Structural modeling of ataxin‐3 reveals distant homology to adaptins
Proteins: Structure, Function, and Bioinformatics, 2002 AbstractSpinocerebellar 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, Hoffmann, Daniel, Evert, Bernd O., Schmitt, Ina, Wüllner, Ullrich, Lengauer, Thomas +5 moreopenaire +4 more sourcesMechanisms 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 sourceMolecular Mechanism of Metal-Ion-Induced Protofibril Formation of Ataxin-3
Bulletin of the Chemical Society of Japan
Ataxin-3, the causative protein of Machado-Joseph disease, comprises an enzymatic domain, Josephin domain (JD), and a polyglutamine repeat (polyQ). The polyQ expansion promotes the fibrillogenesis of ataxin-3, which is responsible for the disease onset.I. Suzuki, Shinya Tahara, Nozomi Goso, T. Morito, Dai Kato, Yusuke Hatakawa, Seon Hwa Lee, Tomoyuki Oe, Kunisato Kuroi, Takakazu Nakabayashi +9 moresemanticscholar +1 more sourceHydrostatic 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 moreopenaire +1 more sourceEvolution 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 (openaire +2 more sourcesBiophysical Characterisation of the Josephin Domain of Ataxin-3
2017 The 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 openaire +1 more source[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 moreopenaire +1 more sourceAtaxin-3 Transgenic Mice
2010 Jean-Michel Scherrmann, Kim Wolff, Christine A. Franco, Marc N. Potenza, Tayfun Uzbay, Lisiane Bizarro, David C. S. Roberts, Robert L. Balster, Sharon L. Walsh, Barbara J. Mason, Charles J. Heyser, Anthony L. Riley, Steve Kohut, Marie-Louise G. Wadenberg, Heather Wilkins, Paul Newhouse, Anne Jackson, Joachim D. Uys, Peter W. Kalivas, Victoria L. Harvey, Tony Dickenson, Daniel Bertrand, Hans Rollema, Raymond S. Hurst, Gorkem Yararbas, Sakire Pogun, Marie-Louise G. Wadenberg, Debby Van Dam, Peter Paul De Deyn, Samuel G. Siris, Richard W. Foltin, Michael J. Kuhar, Robert L. Balster, Sharon L. Walsh, Robert L. Balster, Sharon L. Walsh, Michel Le Moal, Christoph Hiemke, Nicola Simola, Micaela Morelli, Nicola Simola, Micaela Morelli, Nicola Simola, Micaela Morelli, Alyson J. Bond, James J. Strain, R. Andrew Chambers, R. H. De Rijk, E. R. de Kloet, Bankole A. Johnson, Andreas Marneros, Suzanne H. Mitchell, Harriet de Wit, Mark Slifstein, Klaus A. Miczek, Rosa M. M. de Almeida, Emil F. Coccaro, David S. Baldwin, John Atack, Hilde Lavreysen, Rosa M. M. de Almeida, Klaus A. Miczek, Alyson J. Bond, Alfonso Abizaid, Shimon Amir, Samuel G. Siris, Joseph H. Friedman, Theodora Duka, Jelena Nesic, Falk Kiefer, Karl Mann, Bankole A. Johnson, Barbara J. Mason, Charles J. Heyser, Karl Mann, Falk Kiefer, Christopher L. Cunningham, Barbara J. Mason, Charles J. Heyser, Yesne Alici, William Breitbart, Subhash C. Pandey, Karl Mann, Falk Kiefer, Kieran O’Malley, Kieran O’Malley, Mitul A. Mehta, Linda Dykstra, John Atack, Hilde Lavreysen, Debby Van Dam, Peter Paul De Deyn, Holden D. Brown, Michael E. Ragozzino, Brian E. Leonard, Malcolm Lader, Peter J. Flor, Inga D. Neumann, Linda P. Spear, Holden D. Brown, Michael E. Ragozzino, Daniel Hoyer, Martina de Zwaan, Michael J. Owens, Chase H. Bourke, Philip J. Cowen, Pedro L. Delgado, Hiroyuki Uchida, Shitij Kapur, Harriet de Wit, Lawrence H. Price, Malcolm Lader, Husseini K. Manji, Jorge A. Quiroz, Seiya Miyamoto, Francisco Aboitiz, Ximena Carrasco, F. Xavier Castellanos, Michael J. Owens, Chase H. Bourke, Elizabeth C. Warburton, Husseini K. Manji, Jorge A. Quiroz, Linda Dykstra, James H. Woods, Mei-Chuan Ko, Gail Winger, Andrew Young, Jill B. Becker, Helen J. Cassaday, Paul Willner, Maria Isabel Colado, A. Richard Green, Marilyn E. Carroll, Peter A. Santi, Iván Izquierdo, Lia R. Bevilaqua, Martin Cammarota, Alfonso Abizaid, Shimon Amir, Jason C. G. Halford, Arthur Christopoulos, Gregory D. Stewart, Patrick M. Sexton, Sheldon Preskorn, Megan M. Dahmen, Jana Lincoln, Edoardo Spina, Brian E. Leonard, J. Craig Nelson, Meghan M. Grady, Stephen M. Stahl, Thomas R. E. Barnes, Michael M. Morgan, MacDonald J. Christie, Linda Dykstra, Joseph H. Friedman, Marie-Louise G. Wadenberg, Wiepke Cahn, Heleen B. M. Boos, H. D. Postma, Seiya Miyamoto, W. Wolfgang Fleischhacker, Samuel B. Hutton, Peter J. Flor, Inga D. Neumann, Inga D. Neumann, Peter J. Flor, Peter J. Flor, Inga D. Neumann, Grasielle C. Kincheski, Leandro J. Bertoglio, Antonio Pádua Carobrez, Helio Zangrossi, Frederico Guilherme Graeff, James Winslow, Yogita Chudasama, Paul Newhouse, Heather Wilkins, Naheed (Max) Mirza, Shuang Yu, Nuno Sousa, Osborne F. X. Almeida, Osborne F. X. Almeida, Nuno Sousa, Shuang Yu, Osborne F. X. Almeida, Nuno Sousa, Shuang Yu, Tim C. Kirkham, Tim C. Kirkham, Jason C. G. Halford, Ronald F. Mucha, Cecilia J. Hillard, Cecilia J. Hillard, Kim Wolff, Fiona Thomson, Susan Napier, Marie-Louise G. Wadenberg, Craig A. Erickson +199 moreopenaire +1 more source
