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Identification of human proteins that modify misfolding and proteotoxicity of pathogenic ataxin-1.

Petrakis S, Raskó T, Russ J, Friedrich RP, Stroedicke M, Riechers SP, Muehlenberg K, Möller A, Reinhardt A, Vinayagam A, Schaefer MH, Boutros M, Tricoire H, Andrade-Navarro MA, Wanker EE.

PLoS Genet. 2012;8(8):e1002897. doi: 10.1371/journal.pgen.1002897. Epub 2012 Aug 16.


Flanking domain stability modulates the aggregation kinetics of a polyglutamine disease protein.

Saunders HM, Gilis D, Rooman M, Dehouck Y, Robertson AL, Bottomley SP.

Protein Sci. 2011 Oct;20(10):1675-81. doi: 10.1002/pro.698. Epub 2011 Aug 18.


Ataxin-2 mediated cell death is dependent on domains downstream of the polyQ repeat.

Ng H, Pulst SM, Huynh DP.

Exp Neurol. 2007 Dec;208(2):207-15. Epub 2007 Aug 28.


An expanded glutamine repeat destabilizes native ataxin-3 structure and mediates formation of parallel beta -fibrils.

Bevivino AE, Loll PJ.

Proc Natl Acad Sci U S A. 2001 Oct 9;98(21):11955-60. Epub 2001 Sep 25.


Ataxin-1 fusion partners alter polyQ lethality and aggregation.

Rich T, Varadaraj A.

PLoS One. 2007 Oct 10;2(10):e1014.


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.


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.


SUMOylation of the polyglutamine repeat protein, ataxin-1, is dependent on a functional nuclear localization signal.

Riley BE, Zoghbi HY, Orr HT.

J Biol Chem. 2005 Jun 10;280(23):21942-8. Epub 2005 Apr 11.


Characterization of the structure and the amyloidogenic properties of the Josephin domain of the polyglutamine-containing protein ataxin-3.

Masino L, Nicastro G, Menon RP, Dal Piaz F, Calder L, Pastore A.

J Mol Biol. 2004 Dec 3;344(4):1021-35.


Multi-domain misfolding: understanding the aggregation pathway of polyglutamine proteins.

Saunders HM, Bottomley SP.

Protein Eng Des Sel. 2009 Aug;22(8):447-51. doi: 10.1093/protein/gzp033. Epub 2009 Jul 9. Review.


Poly-ubiquitin binding by the polyglutamine disease protein ataxin-3 links its normal function to protein surveillance pathways.

Chai Y, Berke SS, Cohen RE, Paulson HL.

J Biol Chem. 2004 Jan 30;279(5):3605-11. Epub 2003 Nov 5.


Expansion of the polyQ repeat in ataxin-2 alters its Golgi localization, disrupts the Golgi complex and causes cell death.

Huynh DP, Yang HT, Vakharia H, Nguyen D, Pulst SM.

Hum Mol Genet. 2003 Jul 1;12(13):1485-96.


Co-chaperone CHIP promotes aggregation of ataxin-1.

Choi JY, Ryu JH, Kim HS, Park SG, Bae KH, Kang S, Myung PK, Cho S, Park BC, Lee do H.

Mol Cell Neurosci. 2007 Jan;34(1):69-79. Epub 2006 Nov 28.


Amino acid sequences flanking polyglutamine stretches influence their potential for aggregate formation.

Nozaki K, Onodera O, Takano H, Tsuji S.

Neuroreport. 2001 Oct 29;12(15):3357-64.


p80 coilin, a coiled body-specific protein, interacts with ataxin-1, the SCA1 gene product.

Hong S, Ka S, Kim S, Park Y, Kang S.

Biochim Biophys Acta. 2003 May 20;1638(1):35-42.


Research on screening and identification of proteins interacting with ataxin-3.

Shen L, Tang JG, Tang BS, Jiang H, Zhao GH, Xia K, Zhang YH, Cai F, Tan LM, Pan Q.

Zhonghua Yi Xue Yi Chuan Xue Za Zhi. 2005 Jun;22(3):242-7.


The relationship between aggregation and toxicity of polyglutamine-containing ataxin-3 in the intracellular environment of Escherichia coli.

Invernizzi G, Aprile FA, Natalello A, Ghisleni A, Penco A, Relini A, Doglia SM, Tortora P, Regonesi ME.

PLoS One. 2012;7(12):e51890. doi: 10.1371/journal.pone.0051890. Epub 2012 Dec 14.


Polyglutamine-rich suppressors of huntingtin toxicity act upstream of Hsp70 and Sti1 in spatial quality control of amyloid-like proteins.

Wolfe KJ, Ren HY, Trepte P, Cyr DM.

PLoS One. 2014 May 14;9(5):e95914. doi: 10.1371/journal.pone.0095914. eCollection 2014.


The role of protein composition in specifying nuclear inclusion formation in polyglutamine disease.

Chai Y, Wu L, Griffin JD, Paulson HL.

J Biol Chem. 2001 Nov 30;276(48):44889-97. Epub 2001 Sep 25.

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