Paulson HL - Search Results

1

Comparison of spinocerebellar ataxia type 3 mouse models identifies early gain-of-function, cell-autonomous transcriptional changes in oligodendrocytes.

Ramani B, Panwar B, Moore LR, Wang B, Huang R, Guan Y, Paulson HL. Ramani B, et al. Among authors: paulson hl. Hum Mol Genet. 2017 Sep 1;26(17):3362-3374. doi: 10.1093/hmg/ddx224. Hum Mol Genet. 2017. PMID: 28854700 Free PMC article.

2

Evidence for proteasome involvement in polyglutamine disease: localization to nuclear inclusions in SCA3/MJD and suppression of polyglutamine aggregation in vitro.

Chai Y, Koppenhafer SL, Shoesmith SJ, Perez MK, Paulson HL. Chai Y, et al. Among authors: paulson hl. Hum Mol Genet. 1999 Apr;8(4):673-82. doi: 10.1093/hmg/8.4.673. Hum Mol Genet. 1999. PMID: 10072437

3

Ataxin-3 with an altered conformation that exposes the polyglutamine domain is associated with the nuclear matrix.

Perez MK, Paulson HL, Pittman RN. Perez MK, et al. Among authors: paulson hl. Hum Mol Genet. 1999 Dec;8(13):2377-85. doi: 10.1093/hmg/8.13.2377. Hum Mol Genet. 1999. PMID: 10556285

4

CREB-binding protein sequestration by expanded polyglutamine.

McCampbell A, Taylor JP, Taye AA, Robitschek J, Li M, Walcott J, Merry D, Chai Y, Paulson H, Sobue G, Fischbeck KH. McCampbell A, et al. Hum Mol Genet. 2000 Sep 1;9(14):2197-202. doi: 10.1093/hmg/9.14.2197. Hum Mol Genet. 2000. PMID: 10958659

5

Dominantly inherited ataxias: lessons learned from Machado-Joseph disease/spinocerebellar ataxia type 3.

Paulson HL. Paulson HL. Semin Neurol. 2007 Apr;27(2):133-42. doi: 10.1055/s-2007-971172. Semin Neurol. 2007. PMID: 17390258 Review.

6

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.

7

The spinocerebellar ataxias.

Paulson HL. Paulson HL. J Neuroophthalmol. 2009 Sep;29(3):227-37. doi: 10.1097/WNO0b013e3181b416de. J Neuroophthalmol. 2009. PMID: 19726947 Free PMC article. Review.

8

Conditional Niemann-Pick C mice demonstrate cell autonomous Purkinje cell neurodegeneration.

Elrick MJ, Pacheco CD, Yu T, Dadgar N, Shakkottai VG, Ware C, Paulson HL, Lieberman AP. Elrick MJ, et al. Among authors: paulson hl. Hum Mol Genet. 2010 Mar 1;19(5):837-47. doi: 10.1093/hmg/ddp552. Epub 2009 Dec 10. Hum Mol Genet. 2010. PMID: 20007718 Free PMC article.

9

Early autophagic response in a novel knock-in model of Huntington disease.

Heng MY, Duong DK, Albin RL, Tallaksen-Greene SJ, Hunter JM, Lesort MJ, Osmand A, Paulson HL, Detloff PJ. Heng MY, et al. Among authors: paulson hl. Hum Mol Genet. 2010 Oct 1;19(19):3702-20. doi: 10.1093/hmg/ddq285. Epub 2010 Jul 8. Hum Mol Genet. 2010. PMID: 20616151 Free PMC article.

10

The Machado-Joseph disease-associated mutant form of ataxin-3 regulates parkin ubiquitination and stability.

Durcan TM, Kontogiannea M, Thorarinsdottir T, Fallon L, Williams AJ, Djarmati A, Fantaneanu T, Paulson HL, Fon EA. Durcan TM, et al. Among authors: paulson hl. Hum Mol Genet. 2011 Jan 1;20(1):141-54. doi: 10.1093/hmg/ddq452. Epub 2010 Oct 11. Hum Mol Genet. 2011. PMID: 20940148 Free PMC article.

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