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Destabilizing the AXH Tetramer by Mutations: Mechanisms and Potential Antiaggregation Strategies.

Grasso G, Morbiducci U, Massai D, Tuszynski JA, Danani A, Deriu MA.

Biophys J. 2018 Jan 23;114(2):323-330. doi: 10.1016/j.bpj.2017.11.025.


Characterization of the AXH domain of Ataxin-1 using enhanced sampling and functional mode analysis.

Deriu MA, Grasso G, Tuszynski JA, Massai D, Gallo D, Morbiducci U, Danani A.

Proteins. 2016 May;84(5):666-73. doi: 10.1002/prot.25017. Epub 2016 Mar 9.


Protein-protein interactions as a strategy towards protein-specific drug design: the example of ataxin-1.

de Chiara C, Menon RP, Kelly G, Pastore A.

PLoS One. 2013 Oct 14;8(10):e76456. doi: 10.1371/journal.pone.0076456. eCollection 2013.


Self-assembly and conformational heterogeneity of the AXH domain of ataxin-1: an unusual example of a chameleon fold.

de Chiara C, Rees M, Menon RP, Pauwels K, Lawrence C, Konarev PV, Svergun DI, Martin SR, Chen YW, Pastore A.

Biophys J. 2013 Mar 19;104(6):1304-13. doi: 10.1016/j.bpj.2013.01.048. Epub 2013 Mar 19.


Conformational fluctuations of the AXH monomer of Ataxin-1.

Grasso G, Deriu MA, Tuszynski JA, Gallo D, Morbiducci U, Danani A.

Proteins. 2016 Jan;84(1):52-9. doi: 10.1002/prot.24954. Epub 2015 Nov 23.


A key lysine residue in the AXH domain of ataxin-1 is essential for its ubiquitylation.

Kang AR, Park SH, Lee S, Choi DY, Kim KP, Song HK, Hong S, Kang S.

Biochim Biophys Acta. 2015 May;1854(5):356-64. doi: 10.1016/j.bbapap.2015.01.012. Epub 2015 Jan 29.


The AXH domain adopts alternative folds the solution structure of HBP1 AXH.

de Chiara C, Menon RP, Adinolfi S, de Boer J, Ktistaki E, Kelly G, Calder L, Kioussis D, Pastore A.

Structure. 2005 May;13(5):743-53.


The AXH module: an independently folded domain common to ataxin-1 and HBP1.

de Chiara C, Giannini C, Adinolfi S, de Boer J, Guida S, Ramos A, Jodice C, Kioussis D, Pastore A.

FEBS Lett. 2003 Sep 11;551(1-3):107-12.


Kaleidoscopic protein-protein interactions in the life and death of ataxin-1: new strategies against protein aggregation.

de Chiara C, Pastore A.

Trends Neurosci. 2014 Apr;37(4):211-8. doi: 10.1016/j.tins.2014.02.003. Epub 2014 Mar 11. Review.


Structural basis of protein complex formation and reconfiguration by polyglutamine disease protein Ataxin-1 and Capicua.

Kim E, Lu HC, Zoghbi HY, Song JJ.

Genes Dev. 2013 Mar 15;27(6):590-5. doi: 10.1101/gad.212068.112.


Polyglutamine is not all: the functional role of the AXH domain in the ataxin-1 protein.

de Chiara C, Menon RP, Dal Piaz F, Calder L, Pastore A.

J Mol Biol. 2005 Dec 9;354(4):883-93. Epub 2005 Oct 18.


Full length amylin oligomer aggregation: insights from molecular dynamics simulations and implications for design of aggregation inhibitors.

Berhanu WM, Masunov AE.

J Biomol Struct Dyn. 2014;32(10):1651-69. doi: 10.1080/07391102.2013.832635. Epub 2013 Sep 13.


The structure of the AXH domain of spinocerebellar ataxin-1.

Chen YW, Allen MD, Veprintsev DB, Löwe J, Bycroft M.

J Biol Chem. 2004 Jan 30;279(5):3758-65. Epub 2003 Oct 28.


Folding of tetrameric p53: oligomerization and tumorigenic mutations induce misfolding and loss of function.

Lubin DJ, Butler JS, Loh SN.

J Mol Biol. 2010 Jan 29;395(4):705-16. doi: 10.1016/j.jmb.2009.11.013. Epub 2009 Nov 11.


Rational design of mutations that change the aggregation rate of a protein while maintaining its native structure and stability.

Camilloni C, Sala BM, Sormanni P, Porcari R, Corazza A, De Rosa M, Zanini S, Barbiroli A, Esposito G, Bolognesi M, Bellotti V, Vendruscolo M, Ricagno S.

Sci Rep. 2016 May 6;6:25559. doi: 10.1038/srep25559.


Chemical shift assignment of the ataxin-1 AXH domain in complex with a CIC ligand peptide.

de Chiara C, Kelly G, Menon RP, McCormick J, Pastore A.

Biomol NMR Assign. 2014 Oct;8(2):325-7. doi: 10.1007/s12104-013-9509-z. Epub 2013 Jul 14.


Protein Environment: A Crucial Triggering Factor in Josephin Domain Aggregation: The Role of 2,2,2-Trifluoroethanol.

Visentin C, Navarro S, Grasso G, Regonesi ME, Deriu MA, Tortora P, Ventura S.

Int J Mol Sci. 2018 Jul 24;19(8). pii: E2151. doi: 10.3390/ijms19082151.


Characterization of the zebrafish atxn1/axh gene family.

Carlson KM, Melcher L, Lai S, Zoghbi HY, Clark HB, Orr HT.

J Neurogenet. 2009;23(3):313-23. doi: 10.1080/01677060802399976. Epub 2008 Dec 10.


Progress in pathogenesis studies of spinocerebellar ataxia type 1.

Cummings CJ, Orr HT, Zoghbi HY.

Philos Trans R Soc Lond B Biol Sci. 1999 Jun 29;354(1386):1079-81. Review.


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