Paulson HL - Search Results

1

Interaction of the polyglutamine protein ataxin-3 with Rad23 regulates toxicity in Drosophila models of Spinocerebellar Ataxia Type 3.

Sutton JR, Blount JR, Libohova K, Tsou WL, Joshi GS, Paulson HL, Costa MDC, Scaglione KM, Todi SV. Sutton JR, et al. Among authors: paulson hl. Hum Mol Genet. 2017 Apr 15;26(8):1419-1431. doi: 10.1093/hmg/ddx039. Hum Mol Genet. 2017. PMID: 28158474 Free PMC article.

2

The deubiquitinating enzyme ataxin-3, a polyglutamine disease protein, edits Lys63 linkages in mixed linkage ubiquitin chains.

Winborn BJ, Travis SM, Todi SV, Scaglione KM, Xu P, Williams AJ, Cohen RE, Peng J, Paulson HL. Winborn BJ, et al. Among authors: paulson hl. J Biol Chem. 2008 Sep 26;283(39):26436-43. doi: 10.1074/jbc.M803692200. Epub 2008 Jul 3. J Biol Chem. 2008. PMID: 18599482 Free PMC article.

3

Ubiquitination directly enhances activity of the deubiquitinating enzyme ataxin-3.

Todi SV, Winborn BJ, Scaglione KM, Blount JR, Travis SM, Paulson HL. Todi SV, et al. Among authors: paulson hl. EMBO J. 2009 Feb 18;28(4):372-82. doi: 10.1038/emboj.2008.289. Epub 2009 Jan 15. EMBO J. 2009. PMID: 19153604 Free PMC article.

4

Activity and cellular functions of the deubiquitinating enzyme and polyglutamine disease protein ataxin-3 are regulated by ubiquitination at lysine 117.

Todi SV, Scaglione KM, Blount JR, Basrur V, Conlon KP, Pastore A, Elenitoba-Johnson K, Paulson HL. Todi SV, et al. Among authors: paulson hl. J Biol Chem. 2010 Dec 10;285(50):39303-13. doi: 10.1074/jbc.M110.181610. Epub 2010 Oct 13. J Biol Chem. 2010. PMID: 20943656 Free PMC article.

5

Ube2w and ataxin-3 coordinately regulate the ubiquitin ligase CHIP.

Scaglione KM, Zavodszky E, Todi SV, Patury S, Xu P, Rodríguez-Lebrón E, Fischer S, Konen J, Djarmati A, Peng J, Gestwicki JE, Paulson HL. Scaglione KM, et al. Among authors: paulson hl. Mol Cell. 2011 Aug 19;43(4):599-612. doi: 10.1016/j.molcel.2011.05.036. Mol Cell. 2011. PMID: 21855799 Free PMC article.

6

Toward understanding Machado-Joseph disease.

Costa Mdo C, Paulson HL. Costa Mdo C, et al. Among authors: paulson hl. Prog Neurobiol. 2012 May;97(2):239-57. doi: 10.1016/j.pneurobio.2011.11.006. Epub 2011 Nov 23. Prog Neurobiol. 2012. PMID: 22133674 Free PMC article. Review.

7

Dominant negative effect of polyglutamine expansion perturbs normal function of ataxin-3 in neuronal cells.

Neves-Carvalho A, Logarinho E, Freitas A, Duarte-Silva S, Costa Mdo C, Silva-Fernandes A, Martins M, Serra SC, Lopes AT, Paulson HL, Heutink P, Relvas JB, Maciel P. Neves-Carvalho A, et al. Among authors: paulson hl. Hum Mol Genet. 2015 Jan 1;24(1):100-17. doi: 10.1093/hmg/ddu422. Epub 2014 Aug 20. Hum Mol Genet. 2015. PMID: 25143392 Free PMC article.

8

A knockin mouse model of spinocerebellar ataxia type 3 exhibits prominent aggregate pathology and aberrant splicing of the disease gene transcript.

Ramani B, Harris GM, Huang R, Seki T, Murphy GG, Costa Mdo C, Fischer S, Saunders TL, Xia G, McEachin RC, Paulson HL. Ramani B, et al. Among authors: paulson hl. Hum Mol Genet. 2015 Mar 1;24(5):1211-24. doi: 10.1093/hmg/ddu532. Epub 2014 Oct 15. Hum Mol Genet. 2015. PMID: 25320121 Free PMC article.

9

Unbiased screen identifies aripiprazole as a modulator of abundance of the polyglutamine disease protein, ataxin-3.

Costa MDC, Ashraf NS, Fischer S, Yang Y, Schapka E, Joshi G, McQuade TJ, Dharia RM, Dulchavsky M, Ouyang M, Cook D, Sun D, Larsen MJ, Gestwicki JE, Todi SV, Ivanova MI, Paulson HL. Costa MDC, et al. Among authors: paulson hl. Brain. 2016 Nov 1;139(11):2891-2908. doi: 10.1093/brain/aww228. Brain. 2016. PMID: 27645800 Free PMC article.

10

Differential toxicity of ataxin-3 isoforms in Drosophila models of Spinocerebellar Ataxia Type 3.

Johnson SL, Blount JR, Libohova K, Ranxhi B, Paulson HL, Tsou WL, Todi SV. Johnson SL, et al. Among authors: paulson hl. Neurobiol Dis. 2019 Dec;132:104535. doi: 10.1016/j.nbd.2019.104535. Epub 2019 Jul 13. Neurobiol Dis. 2019. PMID: 31310802 Free PMC article.

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