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Publications (Most Recent First)

Maria do Carmo Costa, Naila S. Ashraf, Svetlana Fischer, Yemen Yang, Emily Schapka, Gnanada Joshi, Thomas J. McQuade, Rahil M.Dharia, Mark Dulchavsky, Michelle Ouyang, David Cook, Duxin Sun, Martha J. Larsen, Jason E. Gestwicki, Sokol V. Todi, Magdalena I.Ivanova, Henry L. Paulson. Unbiased screen identifies aripiprazole as a modulator of abundance of the polyglutamine disease protein, ataxin-3. Brain. 2016 Sept 19;139(11):2891-2908. doi: 10.1093/brain/aww288. [Epub ahead of print] PMID: 27645800
Abstract


Zeng L, Wang B, Merillat SA, N Minakawa E, Perkins MD, Ramani B, Tallaksen-Greene SJ, Costa MD, Albin RL, Paulson HL. Differential recruitment of UBQLN2 to nuclear inclusions in the polyglutamine diseases HD and SCA3. Neurobiol Dis. 2015 Jun 30;82:281-288. doi: 10.1016/j.nbd.2015.06.017. [Epub ahead of print] PMID: 26141599

Abstract

  

Atkin G, Moore S, Lu Y, Nelson RF, Tipper N, Rajpal G, Hunt J, Tennant W, Hell JW, Murphy GG,Paulson H. Loss of F-box only protein 2 (Fbxo2) disrupts levels and localization of select NMDA receptor subunits, and promotes aberrant synaptic connectivity. J Neurosci. 2015 Apr 15;35(15):6165-78. doi: 10.1523/JNEUROSCI.3013-14.2015. PMID: 25878288

Abstract

  

Ramani B, Harris GM, Huang R, Seki T, Murphy GG, Costa Mdo C, Fischer S, Saunders TL, Xia G, McEachin RC, Paulson HL. A knockin mouse model of spinocerebellar ataxia type 3 exhibits prominent aggregate pathology and aberrant splicing of the disease gene transcript. Hum Mol Genet. 2015 Mar 1;24(5):1211-24. doi: 10.1093/hmg/ddu532. Epub 2014 Oct 15. PMID: 25320121

Abstract



Atkin G, Paulson H. Ubiquitin pathways in neurodegenerative disease. Front Mol Neurosci. 2014 Jul 8;7:63. doi: 10.3389/fnmol.2014.00063. eCollection 2014. Review. PubMed PMID: 25071440; PubMed Central PMCID: PMC4085722.
Abstract

Rüb U, Hentschel M, Stratmann K, Brunt E, Heinsen H, Seidel K, Bouzrou M, Auburger G, Paulson H, Vonsattel JP, Lange H, Korf HW, den Dunnen W. Huntington's disease (HD): degeneration of select nuclei, widespread occurrence of neuronal nuclear and axonal inclusions in the brainstem. Brain Pathol. 2014 Apr;24(3):247-60. doi: 10.1111/bpa.12115. Epub 2014 Mar 3. PubMed PMID: 24779419..
Abstract

Luna-Cancalon K, Sikora KM, Pappas SS, Singh V, Wulff H, Paulson HL, Burmeister M, Shakkottai VG.Alterations in cerebellar physiology are associated with a stiff-legged gait in Atcay(ji-hes) mice. Neurobiol Dis. 2014 Jul;67:140-8. doi: 10.1016/j.nbd.2014.03.020. Epub 2014 Apr 12. PubMed PMID: 24727095; PubMed Central PMCID: PMC4059535..
Abstract

Atkin G, Hunt J, Minakawa E, Sharkey L, Tipper N, Tennant W, Paulson HL. F-boxonly protein 2 (Fbxo2) regulates amyloid precursor protein levels and processing. J Biol Chem. 2014 Mar 7;289(10):7038-48. doi: 10.1074/jbc.M113.515056. Epub 2014 Jan 27. PubMed PMID: 24469452; PubMed Central PMCID: PMC3945364.

Abstract

Gallagher MD, Suh E, Grossman M, Elman L, McCluskey L, Van Swieten JC, Al-Sarraj S, Neumann M, Gelpi E, Ghetti B, Rohrer JD, Halliday G, Van Broeckhoven C, Seilhean D, Shaw PJ, Frosch MP, Alafuzoff I, Antonell A, Bogdanovic N, Brooks W, Cairns NJ, Cooper-Knock J, Cotman C, Cras P, Cruts M, De Deyn PP, DeCarli C, Dobson-Stone C, Engelborghs S, Fox N, Galasko D, Gearing M, Gijselinck I, Grafman J, Hartikainen P, Hatanpaa KJ, Highley JR, Hodges J, Hulette C, Ince PG, Jin LW, Kirby J, Kofler J, Kril J, Kwok JB, Levey A, Lieberman A, Llado A, Martin JJ, Masliah E, McDermott CJ, McKee A, McLean C, Mead S, Miller CA, Miller J, Munoz DG, Murrell J, Paulson H, Piguet O, Rossor M, Sanchez-Valle R, Sano M, Schneider J, Silbert LC, Spina S, van der Zee J, Van Langenhove T, Warren J, Wharton SB, White CL 3rd, Woltjer RL, Trojanowski JQ, Lee VM, Van Deerlin V, Chen-Plotkin AS. TMEM106B is a genetic modifier of frontotemporal lobar degeneration with C9orf72 hexanucleotide repeat expansions. Acta Neuropathol. 2014 Mar;127(3):407-18. doi: 10.1007/s00401-013-1239-x. Epub 2014 Jan 19. PubMed PMID: 24442578; PubMed Central PMCID: PMC4003885
Abstract

Todd PK, Paulson HL. C9orf72-associated FTD/ALS: when less is more. Neuron. 2013 Oct 16;80(2):257-8. doi: 10.1016/j.neuron.2013.10.010. PubMed PMID: 24139028.
Abstract

Faggiano S, Menon RP, Kelly GP, McCormick J, Todi SV, Scaglione KM, Paulson HL, Pastore A.Enzymatic production of mono-ubiquitinated proteins for structural studies: The example of the Josephin domain of ataxin-3. FEBS Open Bio. 2013 Oct 12;3:453-8. doi: 10.1016/j.fob.2013.10.005. eCollection 2013. PubMed PMID: 24251111; PubMed Central PMCID: PMC3829987
Abstract

Blair LJ, Nordhues BA, Hill SE, Scaglione KM, O'Leary JC 3rd, Fontaine SN, Breydo L, Zhang B, Li P, Wang L, Cotman C, Paulson HL, Muschol M, Uversky VN, Klengel T, Binder EB, Kayed R, Golde TE, Berchtold N, Dickey CA. Accelerated neurodegeneration through chaperone-mediated oligomerization of tau. J Clin Invest. 2013 Oct 1;123(10):4158-69. doi: 10.1172/JCI69003. Epub 2013 Sep 3. PubMed PMID: 23999428; PubMed Central PMCID: PMC3784538
Abstract

Zeng L, Tallaksen-Greene SJ, Wang B, Albin RL, Paulson HL. The de-ubiquitinating enzyme ataxin-3 does not modulate disease progression in a knock-in mouse model of Huntington disease. J Huntingtons Dis. 2013;2(2):201-15. doi: 10.3233/JHD-130058. PubMed PMID: 24683430; PubMed Central PMCID: PMC3966139.
Abstract

do Carmo Costa M, Paulson HL.  New hope for therapy in neurodegenerative diseases.  Cell Res. 2013 Jul 23. doi:10.1038/cr.2013.96. [Epub ahead of print]  PubMed PMID: 23877406
Abstract

Seki T, Gong L, Williams AJ, Sakai N, Todi SV, Paulson HL. JosD1, a membrane-targeted deubiquitinating enzyme, is activated by ubiquitination and regulates membrane dynamics, cell motility, and endocytosis. J Biol Chem. 2013 Jun 14;288(24):17145-55. doi: 10.1074/jbc.M113.463406. Epub 2013 Apr 26. PubMed PMID: 23625928; PubMed Central PMCID: PMC3682520.
Abstract

Smith MC, Scaglione KM, Assimon VA, Patury S, Thompson AD, Dickey CA, Southworth DR, Paulson HL, Gestwicki JE, Zuiderweg ER.  The E3 Ubiquitin Ligase CHIP and the Molecular Chaperone Hsc70 Form a Dynamic, Tethered Complex.  Biochemistry. 2013 Jul 18. [Epub ahead of print]  PubMed PMID: 23865999
Abstract

Rodriguez-Lebron E, do Carmo Costa M, Molina-Luna K, Peron TM, Fischer S, Boudreau RL, Davidson BL, Paulson HL.  Silencing mutant ATXN3 expression resolves molecular phenotypes in SCA3 transgenic mice.  Mol Ther. 2013 Jul 3. doi: 10.1038/mt.2013.152. [Epub ahead of print]  PubMed PMID: 23820820
Abstract

do Carmo Costa M, Luna-Cancalon K, Fischer S, Ashraf NS, Ouyang M, Dharia RM, Martin-Fishman L, Yang Y, Shakkottai VG, Davidson BL, Rodriguez-Lebron E, Paulson HL.  Toward RNAi therapy for the polyglutamine disease Machado-Joseph disease.  Mol Ther, 2013 Jun 14. doi: 10.1038/mt.2013.144. [Epub ahead of print] PubMed PMID: 23765441
Abstract

Scaglione KM, Basrur V, Ashraf NS, Konen JR, Elenitoba-Johnson KS, Todi SV, Paulson HL.  The ubiquitin conjugating enzyme (E2) Ube2x ubiquitinates the N-terminus of substrates.  J Biol Chem.  2013 May 21.  PMID: 23696636
Abstract

Todd PK, Oh SY, Krans A, He F, Sellier C, Frazer M, Renoux AJ, Chen KC, Scaglione KM, Basrur V, Elenitoba-Johnson K, Vonsattel JP, Louis ED, Sutton MA, Taylor JP, Mills RE, Charlet-Berguerand N, Paulson HL.  CGG Repeat-Associated Translation Mediates Neurodegeneration in Fragile X Tremor Ataxia.  Neuron. 2013 May 8;78(3):440-55. doi: 10.1016/j.neuron.2013.03.026. Epub 2013 Apr 18.  PMID: 23602499
Abstract

Seki T, Yoshino K-i, Tanaka S, Dohi E, Onji T, Yamamoto K, Hide I, Paulson HL, Saito N, Sakai N. Establishment of a novel flourescence-based method to evaluate chaperone-mediated autophagy in a single neuron.  PLoS ONE (2012) 7(2): e31232. PMID:22362588
Abstract

Shakkottai VG, do Carmo Costa M, Dell'Orco JM, Sankaranarayanan A, Wulff H, Paulson HL.  Early changes in cerebellar physiology accompany motor dysfunction in the polyglutamine disease spinocerebellar ataxia type 3.J Neurosci. 2011 Sep 7;31(36):13002-14. PMID:21900579
Abstract

Laco MN, Oliviera CR, Paulson HL, Rego AC.  Compromised Mitochondrial Complex II in Models of Machado-Joseph Disease.  Biochim Biophys Acta. 2012 Feb; 1822(2):139-49. PMID:22037589
Abstract

Henry L. Paulson and Roger L. Albin.  Huntington's Disease: Clinical Features and Routes to Therapy. In: Lo DC, Hughes RE, editors.  Neurobiology of Huntington's Disease: Applications to Drug Discovery.  CRC Press; 2011 Chapter 1. PMID:21882418
Abstract

Scaglione KM, Zavodszky E, Todi SV, Patury S, Xu P, Rodriguez-Lebron E. Fischer S, Konen J, Djarmati A, Peng J,  Gestwicki JE, Paulson HL.  Ube2w and ataxin-3 coordinately regulate the ubiquitin ligase CHIP. Mol Cell. 2011 Aug 19;43(4):599-612.  PMID:21855799
Abstract

Todi SV, Paulson HL.  Balancing act: deubiquitinating enzymes in the nervous system. Trends Neurosci. 2011 June 24.  PMID:21704388
Abstract

Costa MD, Paulson HL. Toward understanding Machado-Joseph disease. Prog. Neurobiol. 2011 Nov 23. PubMed PMID:22133674
Abstract

Paulson H. Machado-Joseph disease/spinocerebellar ataxia type 3. Handb Clin Neurol. 2012;103:437-49. Review. PubMed PMID:21827905. 
Abstract

Klockgether T, Paulson H. Milestones in ataxia. Mov Disord. 2011
May;26(6):1134-41. doi: 10.1002/mds.23559. Review. PubMed PMID: 21626557; PMID:3105349.
Abstract

Tsou WL, Soong BW, Paulson HL, Rodríguez-Lebrón E. Splice isoform-specific
suppression of the CaV2.1 variant underlying spinocerebellar ataxia type 6.
Neurobiol Dis. 2011 Sep;43(3):533-42. Epub 2011 Apr 29. PubMed PMID: 21550405; PubMed Central PMCID: 3169420

Abstract

Todd PK, Oh SY, Krans A, Pandey UB, Di Prospero NA, Min KT, Taylor JP, Paulson HL. Histone deacetylases suppress CGG repeat-induced neurodegeneration via transcriptional silencing in models of fragile X tremor ataxia syndrome. PLoS Genet. 2010 Dec 9;6(12):e1001240. PMID: 21170301.
Abstract

Harris GM, Dodelzon K, Gong L, Gonzalez-Alegre P, Paulson HL. Splice isoforms of the polyglutamine disease protein ataxin-3 exhibit similar enzymatic yet different aggregation properties. PLoS One. 2010 Oct 27;5(10):e13695. PMID: 21060878. 
Abstract

Todi SV, Scaglione KM, Blount JR, Basrur V, Conlon KP, Pastore A, Elenitoba-Johnson K, Paulson HL.Activity and cellular functions of the deubiquitinating enzyme and polyglutamine disease protein ataxin-3 are regulated by ubiquitination at lysine 117. J Biol Chem. 2010 Dec 10;285(50):39303-13. Epub 2010 Oct 13. PMID: 20943656. 
Abstract 

Durcan TM, Kontogiannea M, Thorarinsdottir T, Fallon L, Williams AJ, Djarmati A, Fantaneanu T, Paulson HL, Fon EA. The Machado-Joseph disease-associated mutant form of ataxin-3 regulates parkin ubiquitination and stability. Hum Mol Genet. 2011 Jan 1;20(1):141-54. Epub 2010 Oct 11. PMID: 20940148. 
Abstract 

Nicastro G, Todi SV, Karaca E, Bonvin AM, Paulson HL, Pastore A. Understanding the role of the Josephin domain in the PolyUb binding and cleavage properties of ataxin-3. PLoS One. 2010 Aug 26;5(8):e12430. PMID: 20865150. 
Abstract

Heng MY, Duong DK, Albin RL, Tallaksen-Greene SJ, Hunter JM, Lesort MJ, Osmand A, Paulson HL, Detloff PJ. Early autophagic response in a novel knock-in model of Huntington disease. Hum Mol Genet. 2010 Oct 1;19(19):3702-20. Epub 2010 Jul 8. PMID: 20616151. 
Abstract

Todd PK, Paulson HL. RNA-mediated neurodegeneration in repeat expansion disorders. Ann Neurol. 2010 Mar;67(3):291-300. Review. PMID: 20373340. 
Abstract

Elrick MJ, Pacheco CD, Yu T, Dadgar N, Shakkottai VG, Ware C, Paulson HL, Lieberman AP. Conditonal Niemann-Pick C mice demonstrate cell autonomous Purkinje cell neurodegeneration. Hum Mol Genet. 2010 Mar 1;19(5):837-47. PMID: 20007718.
Abstract

D'Abreu A, França MC Jr, Paulson HL, Lopes-Cendes I. Caring for Machado-Joseph disease: current understanding and how to help patients. Parkinsonism Relat Disord. 2010 Jan;16(1):2-7. PMID: 19811945
Abstract

Elrick MJ, Pacheco CD, Yu T, Dadgar N, Shakkottai VG, Ware C, Paulson HL, Lieberman AP. Conditional Niemann-Pick C mice demonstrate cell autonomous Purkinje cell neurodegeneration. Hum Mol Genet. 2009 Dec 24. PMID: 20007718.
Abstract

Paulson HL. The spinocerebellar ataxias. J Neuroophthalmol. 2009 Sep;29(3):227-37. Review. PMID: 19726947.
Abstract

Beglinger LJ, Adams WH, Paulson H, Fiedorowicz JG, Langbehn DR, Duff K, Leserman A, Paulsen JS.Randomized controlled trial of Atomoxetine for cognitive dysfunction in early Huntington disease. J Clin Psychopharm. 2009 Oct;29(5):484-7. PMID:19745649.
Abstract

Saluja I, Paulson H, Gupta A, Turner RS.  X11a haploinsufficiency enhances Ab amyloid deposition in Alzheimer’s disease transgenic mice.  Neurobiol Dis. 2009 Oct;36(1):162-8. PMID: 19631749.
Abstract

Rodríguez-Lebrón E, Gouvion CM, Moore SA, Davidson BL, Paulson H. Allele-specific RNAi leads to neuropathological improvements in a transgenic model of Alzheimer’s disease. Mol Ther. 2009 Sep;17(9):1563-73. PMID: 19532137.
Abstract

Shakkottai VG, Paulson HL. Physiologic alterations in ataxia: Channeling changes into novel therapies.  Arch Neurol. 2009 Oct;66(10):1196-201 PMID: 19822774.
Abstract

Todi SV, Winborn BJ, Scaglione KM, Blount JR, Travis SM, Paulson HL. Ubiquitination directly enhances activity of the deubiquitinating enzyme ataxin-3. EMBO J. 2009 Feb 18;28(4):372-82. PMID: 19153604.
Abstract

Williams AJ, Knutson TM, Colomer Gould VF, Paulson HL.  In vivo suppression of polyglutamine neurotoxicity by C-terminus of Hsp70 interacting protein (CHIP) supports an aggregation model of pathogenesis. Neurobiol Dis. 2009 Mar;33(3):342-53. PMID: 19084066.
Abstract

Huntington Study Group (H Paulson, site investigator at Univ Iowa). Randomized, controlled trial of ethyl-EPA in Huntington disease: The TREND-HD Study.  Arch Neurol 2008 Dec;65(12):1582-9.
Abstract

Huntington Study Group (H Paulson, site investigator at Univ Iowa). Communicating clinical trail results to research participants. Arch Neurol 2008 Dec;65(12):1590-1595.
Abstract

Williams AJ, Paulson HL.  Polyglutamine neurodegeneration: Protein misfolding revisited. Trends Neurosci. 2008 Oct;31(10):521-8. PMID:18778858.
Abstract

Winborn BJ, Travis SM, Todi SV…Paulson HL (9/9).  The deubiquitinating enzyme ataxin-3, a polyglutamine disease protein, edits K63-linkages in mixed linkage ubiquitin chainsJ Biol Chem. 2008 Sept 26;283(39):26436-43. PMID: 18599482
Abstract

Harraz MM, Marden JJ, Zhou W...Paulson H (6/7)...Engelhardt.SOD1/Rac redox sensor regulates NADPH oxidase and dysfunctions in familial ALS model. J Clin Invest. 2008 Feb;118(2):659-70. PMID: 18219391.
Abstract


Duff K, Beglinger LJ, O'Rourke ME...Paulson HL (5/6)...Paulsen JS. Risperidone and the treatment of psychiatric, motor, and cognitive symptoms in Huntington's disease. Ann Clin Psychiatry. 2008 Jan-Mar;20(1):1-3. PMID: 18297579.
Abstract

Glenn KA, Nelson RF, Wen HM, Mallinger AJ, Paulson HL. Diversity in tissue expression, substrate binding and SCF complex formation for a lectin family of ubiquitin ligases. J Biol Chem. 2008 May 9;283(19):12717-29. PMID: 18203720.
Abstract

Gonzalez-Alegre P, Paulson H. Technology insight: Therapeutic RNA interference: how far from the neurology clinic? Nat Clin Pract Neurol. 2007 Jul;3(7):394-404. PMID: 17611488.
Abstract

Nopoulos P, Magnotta VA, Mikos A...Paulson H (4/6)...Paulsen JS. Morphology of the cerebral cortex in preclinical Huntington's disease. Am J Psychiatry. 2007 Sep;164(9):1428-34. PMID: 17728429. 
Abstract

Todi SV, Laco MN, Winborn BJ, Travis SM, Wen HM, Paulson HL. Cellular turnover of the polyglutamine disease protein ataxin-3 is regulated by its catalytic activity. J Biol Chem. 2007 Oct 5;282(40)29348-58. PMID: 17693639. 
Abstract

Marden JJ, Harraz MM, Zhou W...Paulson H (6/7)...Engelhardt J. Redox modifier genes in amyotrophic lateral sclerosis in mice. J Clin Invest. 2007 Oct;117(10):2913-9. PMID: 17853944. 
Abstract

Rodriguez-Lebron E. Paulson H. Allele-specific RNA interference for neurological disease. Gene Ther. 2006 Mar; 13(6):576-81. PMID: 16355113.
Abstract

Nelson RF, GlennKA, Miller VM, WenH, Paulson HL. A novel route for F-box protein mediated ubiquitination links CHIP to glycoprotein quality control. J Biol Chem. 2006 Jul 21; 281(29):20242-51. PMID: 16682404. 
Abstract 

Rub U, de Vos RA, Brunt ER...Paulson H (12/13)...Deller T. Spinocerebellar ataxia Type 3 (SCA3): Thalamic neurodegeneration occurs independently from thalamic ataxin-3 immunopositive neuronal intranuclear inclusions. Brain Pathol, 16(3):218-27, 2006. 
Abstract

Nelson RF, Zhang Y, Zhou Z...PaulsonHL (9/9). Selective cochlear degeneration in mice lacking the F-box protein, Fbx2, a glycoprotein-specific ubiquitin ligase subunit. J Neuroscience. 2007 May 9;27(19):5163-71. PMID: 17494702. 
Abstract

Paulson HL. If it's not one thing, it's another. Nat Genet. 2006 Jul;38(7):743-4. PMID: 16804538.
Abstract

Paulson HL. RNA interference as potential therapy for neurodegenerative disease: applications to inclusion-body myositis? Neurology. 2006 Jan 24;66(2 Suppl 1):S114-7. Review. PMID: 16432138.
Abstract

Paulsen JS, Magnotta VA, Mikos AE, Paulson HL, Penziner E, Andreasen NC, Nopoulos PC. Brain structure in preclinical Huntington's disease. Biol Psychiatry. 2006 Jan 1;59(1):57-63. PMID: 16112655.
Abstract

Miller VM, Paulson HL, Gonzalez-Alegre P. RNA interference in neuroscience: progress and challenges. Cell Mol Neurobiol. 2005 Dec;25(8):1195-207. PMID: 16388332.
Abstract

Gonzalez-Alegre P, Bode N, Davidson BL, Paulson HL. Silencing primary dystonia: lentiviral-mediated RNA interference therapy for DYT1 dystonia. J Neurosci. 2005 Nov 9;25(45):10502-9. PMID: 16280588.
Abstract

Miller VM, Nelson RF, Gouvion CM, Williams A, Rodriguez-Lebron E, Harper SQ, Davidson BL, Rebagliati MR, Paulson HL. CHIP suppresses polyglutamine aggregation and toxicity in vitro and in vivo. J Neurosci. 2005 Oct 5;25(40):9152-61. PMID: 16207874.
Abstract

Berke SJ, Chai Y, Marrs GL, Wen H, Paulson HL. Defining the role of ubiquitin-interacting motifs in the polyglutamine disease protein, ataxin-3. J Biol Chem. 2005 Sep 9;280(36):32026-34. PMID: 16040601.
Abstract

Paulson HL, Miller VM. Breaks in coordination: DNA repair in inherited ataxia. Neuron. 2005 Jun 16;46(6):845-8. Review. PMID: 159534113.
Abstract

Gonzalez-Alegre P, Paulson HL. Aberrant cellular behavior of mutant torsinA implicates nuclear envelope dysfunction in DYT1 dystonia. J Neurosci. 2004 Mar 17;24(11):2593-601. PMID: 15028751. 
Abstract

Shoesmith-Berke SJ, Flores-Schmied FA, Brunt ER, Ellerby LM, Paulson HL. Caspase-mediated proteolysis of the polyglutamine disease protein ataxin-3. J Neurochem. 2004 May; 89(4):908-18. PMID: 15140190.
Abstract

Davidson BL, Paulson HL. Molecular medicine for the brain: silencing of disease genes with RNA interference. Lancet Neurol. 2004 Mar;3(3):145-9. Review. PMID: 14980529.
Abstract

Miller VM, Gouvion CM, Davidson BL, Paulson HL. Targeting Alzheimer's disease genes with RNA interference: an efficient strategy for silencing mutant alleles. Nucleic Acids Res. 2004 Jan 30;32(2):661-8. PMID: 14754988.
Abstract

Chai Y, Shoesmith-Berke S, Cohen RE, Paulson HL.  Poly-ubiquintin binding by the polyglutamine disease protein ataxin-3 links its normal function to protein surveillance pathways. J Biol Chem. 2004 Jan 30;279(5):3605-11. PMID: 14602712.
Abstract

Miller VM, Xia H, Marrs GL, Gouvion CM, Lee G, Davidson BL, Paulson HL. Allele-specific silencing of dominant disease genes. Proc Natl Acad Sci U S A. 2003 Jun 10;100(12):7195-200. PMID: 12782788.
Abstract

Shoesmith-Berke SJ, Paulson HL. Protein aggregation and the ubiquitin proteasome pathway: gaining the UPPer hand on neurodegeneration. Curr Opin Genet Dev. 2003 Jun;13(3):253-61. Review. PMID: 12787787.
Abstract

Gonzalez-Alegre P, Miller VM, Davidson BL, Paulson HL. Toward therapy for DYT1 dystonia: allele-specific silencing of mutant torsinA. Ann Neurol. 2003 Jun;53(6):781-7. PMID: 12783425.
Abstract

Chai Y, Shao J, Miller VM, Williams A, Paulson HL. Live-cell imaging reveals divergent intracellular dynamics of polyglutamine disease proteins and supports a sequestration model of pathogenesis.Proc Natl Acad Sci U S A. 2002 Jul 9;99(14):9310-5. PMID: 12084819.
Abstract

Chai Y, Wu L, Griffin JD, Paulson HL. The role of protein composition in specifying nuclear inclusion formation in polyglutamine disease. J Biol Chem. 2001 Nov 30;276(48):44889-97. PMID: 11572863.
Abstract

Paulson HL, Bonini NM, Roth K. Polyglutamine diseases and neuronal cell death. Proc Natl Acad Sci U S A. 2000 Nov 21;97(24):12957-8. PMID: 11058149.
Abstract 

McCampbell A, Taye AA, Taylor JP, Li M, Walcott J, Merry D, Chai Y, Paulson HL, Sobue G, Fischbeck KH. CREB-binding protein sequestration by expanded polyglutamine. Hum Mol Genet. 2000 Sep 1;9(14):2197-202. PMID: 10958659.
Abstract

Chan HYE, Warrick GL, Gray-Board GL, Koppenhafer SL, Paulson HL, Bonini NM. Mechanisms of chaperone suppression of polyglutamine disease: selectivity, synergy, and modulation of protein solubility in Drosophila. Hum Mol Genet. 2000 Nov 22;9(19):2811-20. PMID: 11092757.
Abstract

Warrick JM., Chan HY, Gray-Board GL, Chai Y, Paulson HL, Bonini NM. Suppression of polyglutamine-mediated neurodegeneration in Drosophila by the molecular chaperone Hsp70. Nat Genet. 1999 Dec;23(4):425-8. PMID: 10581028.
Abstract

Perez MK, Paulson HL, Pittman RN. Ataxin-3 with an altered conformation that exposes the polyglutamine domain is associated with the nuclear matrix. Hum Mol Genet. 1999 Dec;8:2377-85. PMID: 10556285.
Abstract

Chai Y, Koppenhafer SL, Bonini NM, Paulson HL. Analysis of the role of heat shock protein (Hsp): molecular chaperones in polyglutamine disease. J Neurosci. 1999 Dec 1;19(23):10338-47. PMID: 10575031.
Abstract

Chai Y, Koppenhafer SL, Shoesmith SJ, Perez MK, Paulson HL. Evidence for proteasome involvement in polyglutamine disease: localization to nuclear inclusions in SCA3/MJD and suppression of polyglutamine aggregation in vitro. Hum Mol Genet. 1999 Apr;8:673-82. PMID: 10072437.
Abstract

Erhardt JA, Hynicka W, DiBenedetto A, Shen N, Stone N, Paulson HL, Pittman RN. A novel F box protein, NFB42, is highly enriched in neurons and induces growth arrest. J Biol Chem. 1998 Dec 25;273(52):35222-7. PMID: 9857061.
Abstract

Paulson HL, Warrick JM, Gary-Board GL, Bui QT, Fischbeck KH, Pittman RN, Bonini NM. Expanded polyglutamine protein forms nuclear inclusions and causes neural degeneration in Drosophila. Cell. 1998 Jun 12;93:939-49. PMID: 9635424.
Abstract

Paulson HL, Perez MK, Trottier Y, Trojanowski JQ, Subramony SH, Das SS, Big P, Mandel J-L, Fischbeck KH, Pittman RN. Intranuclear Inclusions of Expanded Polyglutamine Protein in SCA3. Neuron. 1997 Aug;19:333-44. PMID: 9292723.
Abstract


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