Henry Paulson, M.D., Ph.D - Publications

Affiliations: 
Neurology University of Michigan, Ann Arbor, Ann Arbor, MI 

183 high-probability publications. We are testing a new system for linking publications to authors. You can help! If you notice any inaccuracies, please sign in and mark papers as correct or incorrect matches. If you identify any major omissions or other inaccuracies in the publication list, please let us know.

Year Citation  Score
2019 Kunkle BW, Grenier-Boley B, Sims R, Bis JC, Damotte V, Naj AC, Boland A, Vronskaya M, van der Lee SJ, Amlie-Wolf A, Bellenguez C, Frizatti A, Chouraki V, Martin ER, Sleegers K, ... ... Paulson HL, et al. Author Correction: Genetic meta-analysis of diagnosed Alzheimer's disease identifies new risk loci and implicates Aβ, tau, immunity and lipid processing. Nature Genetics. PMID 31417202 DOI: 10.1038/s41588-019-0495-7  0.32
2019 Moore LR, Keller L, Bushart DD, Delatorre RG, Li D, McLoughlin HS, do Carmo Costa M, Shakkottai VG, Smith GD, Paulson HL. Antisense oligonucleotide therapy rescues aggresome formation in a novel spinocerebellar ataxia type 3 human embryonic stem cell line. Stem Cell Research. 39: 101504. PMID 31374463 DOI: 10.1016/j.scr.2019.101504  0.32
2019 McKay EC, Beck JS, Khoo SK, Dykema KJ, Cottingham SL, Winn ME, Paulson HL, Lieberman AP, Counts SE. Peri-Infarct Upregulation of the Oxytocin Receptor in Vascular Dementia. Journal of Neuropathology and Experimental Neurology. 78: 436-452. PMID 30990880 DOI: 10.1093/jnen/nlz023  0.32
2019 Lai RY, Tomishon D, Figueroa KP, Pulst SM, Perlman S, Wilmot G, Gomez CM, Schmahmann JD, Paulson H, Shakkottai VG, Ying SH, Zesiewicz T, Bushara K, Geschwind M, Xia G, et al. Tremor in the Degenerative Cerebellum: Towards the Understanding of Brain Circuitry for Tremor. Cerebellum (London, England). PMID 30830673 DOI: 10.1007/s12311-019-01016-6  0.32
2019 Kunkle BW, Grenier-Boley B, Sims R, Bis JC, Damotte V, Naj AC, Boland A, Vronskaya M, van der Lee SJ, Amlie-Wolf A, Bellenguez C, Frizatti A, Chouraki V, Martin ER, Sleegers K, ... ... Paulson HL, et al. Genetic meta-analysis of diagnosed Alzheimer's disease identifies new risk loci and implicates Aβ, tau, immunity and lipid processing. Nature Genetics. 51: 414-430. PMID 30820047 DOI: 10.1038/s41588-019-0358-2  0.32
2018 Sharkey LM, Safren N, Pithadia AS, Gerson JE, Dulchavsky M, Fischer S, Patel R, Lantis G, Ashraf N, Kim JH, Meliki A, Minakawa EN, Barmada SJ, Ivanova MI, Paulson HL. Mutant UBQLN2 promotes toxicity by modulating intrinsic self-assembly. Proceedings of the National Academy of Sciences of the United States of America. PMID 30333186 DOI: 10.1073/pnas.1810522115  0.32
2018 Zeng L, Zhang D, McLoughlin HS, Zalon AJ, Aravind L, Paulson HL. Loss of the Spinocerebellar Ataxia type 3 disease protein ATXN3 alters transcription of multiple signal transduction pathways. Plos One. 13: e0204438. PMID 30231063 DOI: 10.1371/journal.pone.0204438  0.32
2018 McLoughlin HS, Moore LR, Chopra R, Komlo R, McKenzie M, Blumenstein KG, Zhao H, Kordasiewicz HB, Shakkottai VG, Paulson HL. Oligonucleotide therapy mitigates disease in Spinocerebellar Ataxia Type 3 mice. Annals of Neurology. PMID 29908063 DOI: 10.1002/ana.25264  0.32
2017 Figueroa KP, Gan SR, Perlman S, Wilmot G, Gomez CM, Schmahmann J, Paulson H, Shakkottai VG, Ying SH, Zesiewicz T, Bushara K, Geschwind M, Xia G, Subramony SH, Ashizawa T, et al. C9orf72 repeat expansions as genetic modifiers for depression in spinocerebellar ataxias. Movement Disorders : Official Journal of the Movement Disorder Society. PMID 29193335 DOI: 10.1002/mds.27258  0.32
2017 Kuo PH, Gan SR, Wang J, Lo RY, Figueroa KP, Tomishon D, Pulst SM, Perlman S, Wilmot G, Gomez CM, Schmahmann JD, Paulson H, Shakkottai VG, Ying SH, Zesiewicz T, et al. Dystonia and ataxia progression in spinocerebellar ataxias. Parkinsonism & Related Disorders. PMID 29089256 DOI: 10.1016/j.parkreldis.2017.10.007  0.32
2017 Gan SR, Wang J, Figueroa KP, Pulst SM, Tomishon D, Lee D, Perlman S, Wilmot G, Gomez CM, Schmahmann J, Paulson H, Shakkottai VG, Ying SH, Zesiewicz T, Bushara K, et al. Postural Tremor and Ataxia Progression in Spinocerebellar Ataxias. Tremor and Other Hyperkinetic Movements (New York, N.Y.). 7: 492. PMID 29057148 DOI: 10.7916/D8GM8KRH  0.32
2017 Wang B, Zeng L, Merillat SA, Ochaba J, Thompson LM, Barmada SJ, Scaglione KM, Paulson HL. The ubiquitin conjugating enzyme Ube2W regulates solubility of the Huntington's disease protein, huntingtin. Neurobiology of Disease. PMID 28986324 DOI: 10.1016/j.nbd.2017.10.002  0.32
2017 Paulson HL, Shakkottai VG, Clark HB, Orr HT. Polyglutamine spinocerebellar ataxias - from genes to potential treatments. Nature Reviews. Neuroscience. PMID 28855740 DOI: 10.1038/nrn.2017.92  0.32
2017 Sims R, van der Lee SJ, Naj AC, Bellenguez C, Badarinarayan N, Jakobsdottir J, Kunkle BW, Boland A, Raybould R, Bis JC, Martin ER, Grenier-Boley B, Heilmann-Heimbach S, Chouraki V, Kuzma AB, ... ... Paulson HL, et al. Rare coding variants in PLCG2, ABI3, and TREM2 implicate microglial-mediated innate immunity in Alzheimer's disease. Nature Genetics. PMID 28714976 DOI: 10.1038/ng.3916  0.32
2017 Ramani B, Harris GM, Huang R, Seki T, Murphy GG, Carmo Costa MD, 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. Human Molecular Genetics. PMID 28605434 DOI: 10.1093/hmg/ddx176  0.44
2016 Luo L, Wang J, Lo RY, Figueroa KP, Pulst SM, Kuo PH, Perlman S, Wilmot G, Gomez CM, Schmahmann JD, Paulson H, Shakkottai VG, Ying SH, Zesiewicz T, Bushara K, et al. The Initial Symptom and Motor Progression in Spinocerebellar Ataxias. Cerebellum (London, England). PMID 27848087 DOI: 10.1007/s12311-016-0836-3  0.32
2016 Flores BN, Dulchavsky ME, Krans A, Sawaya MR, Paulson HL, Todd PK, Barmada SJ, Ivanova MI. Distinct C9orf72-Associated Dipeptide Repeat Structures Correlate with Neuronal Toxicity. Plos One. 11: e0165084. PMID 27776165 DOI: 10.1371/journal.pone.0165084  0.4
2016 Zhu F, Panwar B, Dodge HH, Li H, Hampstead BM, Albin RL, Paulson HL, Guan Y. COMPASS: A computational model to predict changes in MMSE scores 24-months after initial assessment of Alzheimer's disease. Scientific Reports. 6: 34567. PMID 27703197 DOI: 10.1038/srep34567  0.32
2016 Costa MD, 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, ... ... Paulson HL, et al. Unbiased screen identifies aripiprazole as a modulator of abundance of the polyglutamine disease protein, ataxin-3. Brain : a Journal of Neurology. PMID 27645800 DOI: 10.1093/brain/aww228  0.32
2015 Lo RY, Figueroa KP, Pulst SM, Perlman S, Wilmot G, Gomez C, Schmahmann J, Paulson H, Shakkottai VG, Ying S, Zesiewicz T, Bushara K, Geschwind M, Xia G, Yu JT, et al. Depression and clinical progression in spinocerebellar ataxias. Parkinsonism & Related Disorders. PMID 26644294 DOI: 10.1016/j.parkreldis.2015.11.021  0.32
2015 Wang B, Merillat SA, Vincent M, Huber AK, Basrur V, Mangelberger D, Zeng L, Elenitoba-Johnson K, Miller RA, Irani DN, Dlugosz AA, Schnell S, Scaglione KM, Paulson HL. Loss of the Ubiquitin-conjugating Enzyme Ube2W results in susceptibility to early postnatal lethality and defects in skin, immune and male reproductive systems. The Journal of Biological Chemistry. PMID 26601958 DOI: 10.1074/jbc.M115.676601  1
2015 Zeng L, Wang B, Merillat SA, 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. Neurobiology of Disease. PMID 26141599 DOI: 10.1016/j.nbd.2015.06.017  1
2015 Oh SY, He F, Krans A, Frazer M, Taylor JP, Paulson HL, Todd PK. RAN translation at CGG repeats induces ubiquitin proteasome system impairment in models of fragile X-associated tremor ataxia syndrome. Human Molecular Genetics. 24: 4317-26. PMID 25954027 DOI: 10.1093/hmg/ddv165  1
2015 Legati A, Giovannini D, Nicolas G, López-Sánchez U, Quintáns B, Oliveira JR, Sears RL, Ramos EM, Spiteri E, Sobrido MJ, Carracedo Á, Castro-Fernández C, Cubizolle S, Fogel BL, Goizet C, ... ... Paulson H, et al. Mutations in XPR1 cause primary familial brain calcification associated with altered phosphate export. Nature Genetics. 47: 579-81. PMID 25938945 DOI: 10.1038/ng.3289  1
2015 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. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 35: 6165-78. PMID 25878288 DOI: 10.1523/JNEUROSCI.3013-14.2015  1
2015 Lo RY, Figueroa KP, Pulst SM, Lin CY, Perlman S, Wilmot G, Gomez CM, Schmahmann J, Paulson H, Shakkottai VG, Ying SH, Zesiewicz T, Bushara K, Geschwind M, Xia G, et al. Vascular risk factors and clinical progression in spinocerebellar ataxias. Tremor and Other Hyperkinetic Movements (New York, N.Y.). 5: 287. PMID 25713748 DOI: 10.7916/D89885S0  0.32
2015 Lo RY, Figueroa KP, Pulst SM, Lin CY, Perlman S, Wilmot G, Gomez C, Schmahmann J, Paulson H, Shakkottai VG, Ying S, Zesiewicz T, Bushara K, Geschwind M, Xia G, et al. Coenzyme Q10 and spinocerebellar ataxias. Movement Disorders : Official Journal of the Movement Disorder Society. 30: 214-20. PMID 25449974 DOI: 10.1002/mds.26088  1
2015 Vittal V, Shi L, Wenzel DM, Scaglione KM, Duncan ED, Basrur V, Elenitoba-Johnson KS, Baker D, Paulson HL, Brzovic PS, Klevit RE. Intrinsic disorder drives N-terminal ubiquitination by Ube2w. Nature Chemical Biology. 11: 83-9. PMID 25436519 DOI: 10.1038/nchembio.1700  1
2015 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. Human Molecular Genetics. 24: 1211-24. PMID 25320121 DOI: 10.1093/hmg/ddu532  0.44
2015 Moscovich M, Okun MS, Favilla C, Figueroa KP, Pulst SM, Perlman S, Wilmot G, Gomez C, Schmahmann J, Paulson H, Shakkottai V, Ying S, Zesiewicz T, Kuo SH, Mazzoni P, et al. Clinical evaluation of eye movements in spinocerebellar ataxias: a prospective multicenter study. Journal of Neuro-Ophthalmology : the Official Journal of the North American Neuro-Ophthalmology Society. 35: 16-21. PMID 25259863 DOI: 10.1097/WNO.0000000000000167  1
2015 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. Dominant negative effect of polyglutamine expansion perturbs normal function of ataxin-3 in neuronal cells. Human Molecular Genetics. 24: 100-17. PMID 25143392 DOI: 10.1093/hmg/ddu422  1
2014 Tallaksen-Greene SJ, Sadagurski M, Zeng L, Mauch R, Perkins M, Banduseela VC, Lieberman AP, Miller RA, Paulson HL, Albin RL. Differential effects of delayed aging on phenotype and striatal pathology in a murine model of Huntington disease. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 34: 15658-68. PMID 25411494 DOI: 10.1523/JNEUROSCI.1830-14.2014  1
2014 Naj AC, Jun G, Reitz C, Kunkle BW, Perry W, Park YS, Beecham GW, Rajbhandary RA, Hamilton-Nelson KL, Wang LS, Kauwe JS, Huentelman MJ, Myers AJ, Bird TD, Boeve BF, ... ... Paulson HL, et al. Effects of multiple genetic loci on age at onset in late-onset Alzheimer disease: a genome-wide association study. Jama Neurology. 71: 1394-404. PMID 25199842 DOI: 10.1001/jamaneurol.2014.1491  0.32
2014 Atkin G, Paulson H. Ubiquitin pathways in neurodegenerative disease. Frontiers in Molecular Neuroscience. 7: 63. PMID 25071440 DOI: 10.3389/fnmol.2014.00063  1
2014 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 Pathology (Zurich, Switzerland). 24: 247-60. PMID 24779419 DOI: 10.1111/bpa.12115  1
2014 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. Neurobiology of Disease. 67: 140-8. PMID 24727095 DOI: 10.1016/j.nbd.2014.03.020  1
2014 Atkin G, Hunt J, Minakawa E, Sharkey L, Tipper N, Tennant W, Paulson HL. F-box only protein 2 (Fbxo2) regulates amyloid precursor protein levels and processing. The Journal of Biological Chemistry. 289: 7038-48. PMID 24469452 DOI: 10.1074/jbc.M113.515056  1
2014 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, ... ... Paulson H, et al. TMEM106B is a genetic modifier of frontotemporal lobar degeneration with C9orf72 hexanucleotide repeat expansions. Acta Neuropathologica. 127: 407-18. PMID 24442578 DOI: 10.1007/s00401-013-1239-x  1
2014 Todd PK, Ackall FY, Hur J, Sharma K, Paulson HL, Dowling JJ. Transcriptional changes and developmental abnormalities in a zebrafish model of myotonic dystrophy type 1. Disease Models & Mechanisms. 7: 143-55. PMID 24092878 DOI: 10.1242/dmm.012427  1
2013 Rodríguez-Lebrón E, Costa MD, Luna-Cancalon K, Peron TM, Fischer S, Boudreau RL, Davidson BL, Paulson HL. Silencing Mutant ATXN3 Expression Resolves Molecular Phenotypes in SCA3 Transgenic Mice. Molecular Therapy : the Journal of the American Society of Gene Therapy. 21: 1909-1918. PMID 28157530 DOI: 10.1038/mt.2013.152  0.6
2013 do Carmo Costa M, Luna-Cancalon K, Fischer S, Ashraf NS, Ouyang M, Dharia RM, Martin-Fishman L, Yang Y, Shakkottai VG, Davidson BL, Rodríguez-Lebrón E, Paulson HL. Toward RNAi Therapy for the Polyglutamine Disease Machado-Joseph Disease. Molecular Therapy : the Journal of the American Society of Gene Therapy. 21: 1898-1908. PMID 28157529 DOI: 10.1038/mt.2013.144  0.6
2013 Jensen FE, Paulson HL, Bar-Or A. Considering a career in neurology research? Heed these words. Annals of Neurology. 74: A12. PMID 25813981 DOI: 10.1002/ana.24008  1
2013 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. Journal of Huntington's Disease. 2: 201-15. PMID 24683430 DOI: 10.3233/JHD-130058  1
2013 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. 3: 453-8. PMID 24251111 DOI: 10.1016/j.fob.2013.10.005  1
2013 Ashizawa T, Figueroa KP, Perlman SL, Gomez CM, Wilmot GR, Schmahmann JD, Ying SH, Zesiewicz TA, Paulson HL, Shakkottai VG, Bushara KO, Kuo SH, Geschwind MD, Xia G, Mazzoni P, et al. Clinical characteristics of patients with spinocerebellar ataxias 1, 2, 3 and 6 in the US; a prospective observational study. Orphanet Journal of Rare Diseases. 8: 177. PMID 24225362 DOI: 10.1186/1750-1172-8-177  1
2013 Todd PK, Paulson HL. C9orf72-associated FTD/ALS: when less is more. Neuron. 80: 257-8. PMID 24139028 DOI: 10.1016/j.neuron.2013.10.010  1
2013 Blair LJ, Nordhues BA, Hill SE, Scaglione KM, O'Leary JC, Fontaine SN, Breydo L, Zhang B, Li P, Wang L, Cotman C, Paulson HL, Muschol M, Uversky VN, Klengel T, et al. Accelerated neurodegeneration through chaperone-mediated oligomerization of tau. The Journal of Clinical Investigation. 123: 4158-69. PMID 23999428 DOI: 10.1172/JCI69003  1
2013 do Carmo Costa M, Paulson HL. New hope for therapy in neurodegenerative diseases. Cell Research. 23: 1159-60. PMID 23877406 DOI: 10.1038/cr.2013.96  1
2013 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. 52: 5354-64. PMID 23865999 DOI: 10.1021/bi4009209  1
2013 Rodríguez-Lebrón E, Costa Mdo C, Costa Md, Luna-Cancalon K, Peron TM, Fischer S, Boudreau RL, Davidson BL, Paulson HL. Silencing mutant ATXN3 expression resolves molecular phenotypes in SCA3 transgenic mice. Molecular Therapy : the Journal of the American Society of Gene Therapy. 21: 1909-18. PMID 23820820 DOI: 10.1038/mt.2013.152  1
2013 Costa Mdo C, Luna-Cancalon K, Fischer S, Ashraf NS, Ouyang M, Dharia RM, Martin-Fishman L, Yang Y, Shakkottai VG, Davidson BL, Rodríguez-Lebrón E, Paulson HL. Toward RNAi therapy for the polyglutamine disease Machado-Joseph disease. Molecular Therapy : the Journal of the American Society of Gene Therapy. 21: 1898-908. PMID 23765441 DOI: 10.1038/mt.2013.144  1
2013 Scaglione KM, Basrur V, Ashraf NS, Konen JR, Elenitoba-Johnson KS, Todi SV, Paulson HL. The ubiquitin-conjugating enzyme (E2) Ube2w ubiquitinates the N terminus of substrates. The Journal of Biological Chemistry. 288: 18784-8. PMID 23696636 DOI: 10.1074/jbc.C113.477596  1
2013 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. The Journal of Biological Chemistry. 288: 17145-55. PMID 23625928 DOI: 10.1074/jbc.M113.463406  1
2013 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, ... ... Paulson HL, et al. CGG repeat-associated translation mediates neurodegeneration in fragile X tremor ataxia syndrome. Neuron. 78: 440-55. PMID 23602499 DOI: 10.1016/j.neuron.2013.03.026  1
2013 Rüb U, Schöls L, Paulson H, Auburger G, Kermer P, Jen JC, Seidel K, Korf HW, Deller T. Clinical features, neurogenetics and neuropathology of the polyglutamine spinocerebellar ataxias type 1, 2, 3, 6 and 7. Progress in Neurobiology. 104: 38-66. PMID 23438480 DOI: 10.1016/j.pneurobio.2013.01.001  1
2013 Hsu SC, Sears RL, Lemos RR, Quintáns B, Huang A, Spiteri E, Nevarez L, Mamah C, Zatz M, Pierce KD, Fullerton JM, Adair JC, Berner JE, Bower M, Brodaty H, ... ... Paulson H, et al. Mutations in SLC20A2 are a major cause of familial idiopathic basal ganglia calcification. Neurogenetics. 14: 11-22. PMID 23334463 DOI: 10.1007/s10048-012-0349-2  1
2013 Rüb U, Hoche F, Brunt ER, Heinsen H, Seidel K, Del Turco D, Paulson HL, Bohl J, von Gall C, Vonsattel JP, Korf HW, den Dunnen WF. Degeneration of the cerebellum in Huntington's disease (HD): possible relevance for the clinical picture and potential gateway to pathological mechanisms of the disease process. Brain Pathology (Zurich, Switzerland). 23: 165-77. PMID 22925167 DOI: 10.1111/j.1750-3639.2012.00629.x  1
2012 França MC, Emmel VE, D'Abreu A, Maurer-Morelli CV, Secolin R, Bonadia LC, da Silva MS, Nucci A, Jardim LB, Saraiva-Pereira ML, Marques W, Paulson H, Lopes-Cendes I. Normal ATXN3 Allele but Not CHIP Polymorphisms Modulates Age at Onset in Machado-Joseph Disease. Frontiers in Neurology. 3: 164. PMID 23181052 DOI: 10.3389/fneur.2012.00164  1
2012 Laço MN, Cortes L, Travis SM, Paulson HL, Rego AC. Valosin-containing protein (VCP/p97) is an activator of wild-type ataxin-3. Plos One. 7: e43563. PMID 22970133 DOI: 10.1371/journal.pone.0043563  1
2012 Gordon KL, Glenn KA, Bode N, Wen HM, Paulson HL, Gonzalez-Alegre P. The ubiquitin ligase F-box/G-domain protein 1 promotes the degradation of the disease-linked protein torsinA through the ubiquitin-proteasome pathway and macroautophagy. Neuroscience. 224: 160-71. PMID 22917612 DOI: 10.1016/j.neuroscience.2012.08.023  1
2012 Todd PK, Paulson HL. Drug discovery: Kill the messenger where it lives. Nature. 488: 36-8. PMID 22859197 DOI: 10.1038/488036a  1
2012 Thompson AD, Scaglione KM, Prensner J, Gillies AT, Chinnaiyan A, Paulson HL, Jinwal UK, Dickey CA, Gestwicki JE. Analysis of the tau-associated proteome reveals that exchange of Hsp70 for Hsp90 is involved in tau degradation. Acs Chemical Biology. 7: 1677-86. PMID 22769591 DOI: 10.1021/cb3002599  1
2012 Tada M, Coon EA, Osmand AP, Kirby PA, Martin W, Wieler M, Shiga A, Shirasaki H, Tada M, Makifuchi T, Yamada M, Kakita A, Nishizawa M, Takahashi H, Paulson HL. Coexistence of Huntington's disease and amyotrophic lateral sclerosis: a clinicopathologic study. Acta Neuropathologica. 124: 749-60. PMID 22735976 DOI: 10.1007/s00401-012-1005-5  1
2012 Broglio SP, Eckner JT, Paulson HL, Kutcher JS. Cognitive decline and aging: the role of concussive and subconcussive impacts. Exercise and Sport Sciences Reviews. 40: 138-44. PMID 22728452 DOI: 10.1097/JES.0b013e3182524273  1
2012 Bakulski KM, Dolinoy DC, Sartor MA, Paulson HL, Konen JR, Lieberman AP, Albin RL, Hu H, Rozek LS. Genome-wide DNA methylation differences between late-onset Alzheimer's disease and cognitively normal controls in human frontal cortex. Journal of Alzheimer's Disease : Jad. 29: 571-88. PMID 22451312 DOI: 10.3233/JAD-2012-111223  1
2012 Seki T, Yoshino KI, Tanaka S, Dohi E, Onji T, Yamamoto K, Hide I, Paulson HL, Saito N, Sakai N. Establishment of a novel fluorescence-based method to evaluate chaperone-mediated autophagy in a single neuron. Plos One. 7: e31232. PMID 22363588 DOI: 10.1371/journal.pone.0031232  1
2012 Bakulski KM, Rozek LS, Dolinoy DC, Paulson HL, Hu H. Alzheimer's disease and environmental exposure to lead: the epidemiologic evidence and potential role of epigenetics. Current Alzheimer Research. 9: 563-73. PMID 22272628  1
2012 Costa Mdo C, Paulson HL. Toward understanding Machado-Joseph disease. Progress in Neurobiology. 97: 239-57. PMID 22133674 DOI: 10.1016/j.pneurobio.2011.11.006  1
2012 Laço MN, Oliveira CR, Paulson HL, Rego AC. Compromised mitochondrial complex II in models of Machado-Joseph disease. Biochimica Et Biophysica Acta. 1822: 139-49. PMID 22037589 DOI: 10.1016/j.bbadis.2011.10.010  1
2012 Paulson H. Machado-Joseph disease/spinocerebellar ataxia type 3. Handbook of Clinical Neurology. 103: 437-49. PMID 21827905 DOI: 10.1016/B978-0-444-51892-7.00027-9  1
2012 Williams AJ, Knutson TM, Colomer Gould VF, Paulson HL. Corrigendum to "In vivo suppression of polyglutamine neurotoxicity by C-terminus of Hsp70-interacting protein (CHIP) supports an aggregation model of pathogenesis"[Neurobiology of Disease 33/3 (2009) 342-353] Neurobiology of Disease. 46: 503. DOI: 10.1016/j.nbd.2012.02.009  1
2011 Paulson HL, Igo I. Genetics of dementia. Seminars in Neurology. 31: 449-60. PMID 22266883 DOI: 10.1055/s-0031-1299784  1
2011 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. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 31: 13002-14. PMID 21900579 DOI: 10.1523/JNEUROSCI.2789-11.2011  1
2011 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. Ube2w and ataxin-3 coordinately regulate the ubiquitin ligase CHIP. Molecular Cell. 43: 599-612. PMID 21855799 DOI: 10.1016/j.molcel.2011.05.036  1
2011 Todi SV, Paulson HL. Balancing act: deubiquitinating enzymes in the nervous system. Trends in Neurosciences. 34: 370-82. PMID 21704388 DOI: 10.1016/j.tins.2011.05.004  1
2011 Klockgether T, Paulson H. Milestones in ataxia. Movement Disorders : Official Journal of the Movement Disorder Society. 26: 1134-41. PMID 21626557 DOI: 10.1002/mds.23559  1
2011 Tsou WL, Soong BW, Paulson HL, Rodríguez-Lebrón E. Splice isoform-specific suppression of the Cav2.1 variant underlying spinocerebellar ataxia type 6. Neurobiology of Disease. 43: 533-42. PMID 21550405 DOI: 10.1016/j.nbd.2011.04.016  1
2011 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. Human Molecular Genetics. 20: 141-54. PMID 20940148 DOI: 10.1093/hmg/ddq452  1
2010 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 Genetics. 6: e1001240. PMID 21170301 DOI: 10.1371/journal.pgen.1001240  1
2010 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. 5: e13695. PMID 21060878 DOI: 10.1371/journal.pone.0013695  1
2010 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. The Journal of Biological Chemistry. 285: 39303-13. PMID 20943656 DOI: 10.1074/jbc.M110.181610  1
2010 Heng MY, Detloff PJ, Paulson HL, Albin RL. Early alterations of autophagy in Huntington disease-like mice. Autophagy. 6: 1206-8. PMID 20935460 DOI: 10.4161/auto.6.8.13617  1
2010 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. 5: e12430. PMID 20865150 DOI: 10.1371/journal.pone.0012430  1
2010 do Carmo Costa M, Bajanca F, Rodrigues AJ, Tomé RJ, Corthals G, Macedo-Ribeiro S, Paulson HL, Logarinho E, Maciel P. Ataxin-3 plays a role in mouse myogenic differentiation through regulation of integrin subunit levels. Plos One. 5: e11728. PMID 20668528 DOI: 10.1371/journal.pone.0011728  1
2010 Seidel K, den Dunnen WF, Schultz C, Paulson H, Frank S, de Vos RA, Brunt ER, Deller T, Kampinga HH, Rüb U. Axonal inclusions in spinocerebellar ataxia type 3. Acta Neuropathologica. 120: 449-60. PMID 20635090 DOI: 10.1007/s00401-010-0717-7  1
2010 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. Human Molecular Genetics. 19: 3702-20. PMID 20616151 DOI: 10.1093/hmg/ddq285  1
2010 Todd PK, Paulson HL. RNA-mediated neurodegeneration in repeat expansion disorders. Annals of Neurology. 67: 291-300. PMID 20373340 DOI: 10.1002/ana.21948  1
2010 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. Human Molecular Genetics. 19: 837-47. PMID 20007718 DOI: 10.1093/hmg/ddp552  1
2010 D'Abreu A, França MC, Paulson HL, Lopes-Cendes I. Caring for Machado-Joseph disease: current understanding and how to help patients. Parkinsonism & Related Disorders. 16: 2-7. PMID 19811945 DOI: 10.1016/j.parkreldis.2009.08.012  1
2009 Shakkottai VG, Paulson HL. Physiologic alterations in ataxia: channeling changes into novel therapies. Archives of Neurology. 66: 1196-201. PMID 19822774 DOI: 10.1001/archneurol.2009.212  1
2009 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. Journal of Clinical Psychopharmacology. 29: 484-7. PMID 19745649 DOI: 10.1097/JCP.0b013e3181b2ac0a  1
2009 Paulson HL. The spinocerebellar ataxias. Journal of Neuro-Ophthalmology : the Official Journal of the North American Neuro-Ophthalmology Society. 29: 227-37. PMID 19726947 DOI: 10.1097/WNO0b013e3181b416de  1
2009 Saluja I, Paulson H, Gupta A, Turner RS. X11alpha haploinsufficiency enhances Abeta amyloid deposition in Alzheimer's disease transgenic mice. Neurobiology of Disease. 36: 162-8. PMID 19631749 DOI: 10.1016/j.nbd.2009.07.006  1
2009 Rodríguez-Lebrón E, Gouvion CM, Moore SA, Davidson BL, Paulson HL. Allele-specific RNAi mitigates phenotypic progression in a transgenic model of Alzheimer's disease. Molecular Therapy : the Journal of the American Society of Gene Therapy. 17: 1563-73. PMID 19532137 DOI: 10.1038/mt.2009.123  1
2009 Todi SV, Winborn BJ, Scaglione KM, Blount JR, Travis SM, Paulson HL. Ubiquitination directly enhances activity of the deubiquitinating enzyme ataxin-3. The Embo Journal. 28: 372-82. PMID 19153604 DOI: 10.1038/emboj.2008.289  1
2009 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. Neurobiology of Disease. 33: 342-53. PMID 19084066 DOI: 10.1016/j.nbd.2008.10.016  1
2008 Beglinger LJ, Paulsen JS, Watson DB, Wang C, Duff K, Langbehn DR, Moser DJ, Paulson HL, Aylward EH, Carlozzi NE, Queller S, Stout JC. Obsessive and compulsive symptoms in prediagnosed Huntington's disease. The Journal of Clinical Psychiatry. 69: 1758-65. PMID 19012814  1
2008 Williams AJ, Paulson HL. Polyglutamine neurodegeneration: protein misfolding revisited. Trends in Neurosciences. 31: 521-8. PMID 18778858 DOI: 10.1016/j.tins.2008.07.004  1
2008 Winborn BJ, Travis SM, Todi SV, Scaglione KM, Xu P, Williams AJ, Cohen RE, Peng J, Paulson HL. The deubiquitinating enzyme ataxin-3, a polyglutamine disease protein, edits Lys63 linkages in mixed linkage ubiquitin chains. The Journal of Biological Chemistry. 283: 26436-43. PMID 18599482 DOI: 10.1074/jbc.M803692200  1
2008 Duff K, Beglinger LJ, O'Rourke ME, Nopoulos P, Paulson HL, Paulsen JS. Risperidone and the treatment of psychiatric, motor, and cognitive symptoms in Huntington's disease. Annals of Clinical Psychiatry : Official Journal of the American Academy of Clinical Psychiatrists. 20: 1-3. PMID 18297579 DOI: 10.1080/10401230701844802  1
2008 Harraz MM, Marden JJ, Zhou W, Zhang Y, Williams A, Sharov VS, Nelson K, Luo M, Paulson H, Schöneich C, Engelhardt JF. SOD1 mutations disrupt redox-sensitive Rac regulation of NADPH oxidase in a familial ALS model. The Journal of Clinical Investigation. 118: 659-70. PMID 18219391 DOI: 10.1172/JCI34060  1
2008 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. The Journal of Biological Chemistry. 283: 12717-29. PMID 18203720 DOI: 10.1074/jbc.M709508200  1
2007 Marden JJ, Harraz MM, Williams AJ, Nelson K, Luo M, Paulson H, Engelhardt JF. Redox modifier genes in amyotrophic lateral sclerosis in mice. The Journal of Clinical Investigation. 117: 2913-9. PMID 17853944 DOI: 10.1172/JCI31265  1
2007 Nopoulos P, Magnotta VA, Mikos A, Paulson H, Andreasen NC, Paulsen JS. Morphology of the cerebral cortex in preclinical Huntington's disease. The American Journal of Psychiatry. 164: 1428-34. PMID 17728429 DOI: 10.1176/appi.ajp.2007.06081266  1
2007 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. The Journal of Biological Chemistry. 282: 29348-58. PMID 17693639 DOI: 10.1074/jbc.M704126200  1
2007 Soong BW, Paulson HL. Spinocerebellar ataxias: an update. Current Opinion in Neurology. 20: 438-46. PMID 17620880 DOI: 10.1097/WCO.0b013e3281fbd3dd  1
2007 Gonzalez-Alegre P, Paulson HL. Technology insight: therapeutic RNA interference--how far from the neurology clinic? Nature Clinical Practice. Neurology. 3: 394-404. PMID 17611488 DOI: 10.1038/ncpneuro0551  1
2007 Langbehn DR, Paulsen JS. Predictors of diagnosis in Huntington disease. Neurology. 68: 1710-7. PMID 17502553 DOI: 10.1212/01.wnl.0000261918.90053.96  1
2007 Nelson RF, Glenn KA, Zhang Y, Wen H, Knutson T, Gouvion CM, Robinson BK, Zhou Z, Yang B, Smith RJ, Paulson HL. Selective cochlear degeneration in mice lacking the F-box protein, Fbx2, a glycoprotein-specific ubiquitin ligase subunit. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 27: 5163-71. PMID 17494702 DOI: 10.1523/JNEUROSCI.0206-07.2007  1
2007 Todi SV, Paulson HL. Dangerous liaisons: polyglutamine meets HMGB. Nature Cell Biology. 9: 359-61. PMID 17401384 DOI: 10.1038/ncb0407-359  1
2007 Paulson HL. Dominantly inherited ataxias: lessons learned from Machado-Joseph disease/spinocerebellar ataxia type 3. Seminars in Neurology. 27: 133-42. PMID 17390258 DOI: 10.1055/s-2007-971172  1
2007 Paulson HL, Pulst SM. Chapter 5 Polyglutamine Ataxias: In Vitro and In Vivo Models Blue Books of Neurology. 31: 145-169. DOI: 10.1016/S1877-184X(09)70079-6  1
2007 Todi SV, Williams AJ, Paulson HL. Polyglutamine Disorders Including Huntington's Disease Molecular Neurology. 257-275. DOI: 10.1016/B978-012369509-3.50019-6  1
2006 Paulson H, Gonzalez-Alegre P. RNAi gets its prize. The Lancet. Neurology. 5: 997-9. PMID 17110276 DOI: 10.1016/S1474-4422(06)70609-3  1
2006 Rüb U, Brunt ER, Petrasch-Parwez E, Schöls L, Theegarten D, Auburger G, Seidel K, Schultz C, Gierga K, Paulson H, van Broeckhoven C, Deller T, de Vos RA. Degeneration of ingestion-related brainstem nuclei in spinocerebellar ataxia type 2, 3, 6 and 7. Neuropathology and Applied Neurobiology. 32: 635-49. PMID 17083478 DOI: 10.1111/j.1365-2990.2006.00772.x  1
2006 Rüb U, de Vos RA, Brunt ER, Sebestény T, Schöls L, Auburger G, Bohl J, Ghebremedhin E, Gierga K, Seidel K, den Dunnen W, Heinsen H, Paulson H, Deller T. Spinocerebellar ataxia type 3 (SCA3): thalamic neurodegeneration occurs independently from thalamic ataxin-3 immunopositive neuronal intranuclear inclusions. Brain Pathology (Zurich, Switzerland). 16: 218-27. PMID 16911479 DOI: 10.1111/j.1750-3639.2006.00022.x  1
2006 Paulson HL. If it's not one thing, it's another. Nature Genetics. 38: 743-4. PMID 16804538 DOI: 10.1038/ng0706-743  1
2006 Nelson RF, Glenn KA, Miller VM, Wen H, Paulson HL. A novel route for F-box protein-mediated ubiquitination links CHIP to glycoprotein quality control. The Journal of Biological Chemistry. 281: 20242-51. PMID 16682404 DOI: 10.1074/jbc.M602423200  1
2006 Paulson H. RNA interference as potential therapy for neurodegenerative disease: applications to inclusion-body myositis? Neurology. 66: S114-7. PMID 16432138 DOI: 10.1212/01.wnl.0000192310.07041.53  1
2006 Rodriguez-Lebron E, Paulson HL. Allele-specific RNA interference for neurological disease. Gene Therapy. 13: 576-81. PMID 16355113 DOI: 10.1038/sj.gt.3302702  1
2006 Paulsen JS, Magnotta VA, Mikos AE, Paulson HL, Penziner E, Andreasen NC, Nopoulos PC. Brain structure in preclinical Huntington's disease. Biological Psychiatry. 59: 57-63. PMID 16112655 DOI: 10.1016/j.biopsych.2005.06.003  1
2005 Miller VM, Paulson HL, Gonzalez-Alegre P. RNA interference in neuroscience: progress and challenges. Cellular and Molecular Neurobiology. 25: 1195-207. PMID 16388332 DOI: 10.1007/s10571-005-8447-4  1
2005 Gonzalez-Alegre P, Bode N, Davidson BL, Paulson HL. Silencing primary dystonia: lentiviral-mediated RNA interference therapy for DYT1 dystonia. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 25: 10502-9. PMID 16280588 DOI: 10.1523/JNEUROSCI.3016-05.2005  1
2005 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. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 25: 9152-61. PMID 16207874 DOI: 10.1523/JNEUROSCI.3001-05.2005  1
2005 Berke SJ, Chai Y, Marrs GL, Wen H, Paulson HL. Defining the role of ubiquitin-interacting motifs in the polyglutamine disease protein, ataxin-3. The Journal of Biological Chemistry. 280: 32026-34. PMID 16040601 DOI: 10.1074/jbc.M506084200  1
2005 Paulson HL, Miller VM. Breaks in coordination: DNA repair in inherited ataxia. Neuron. 46: 845-8. PMID 15953413 DOI: 10.1016/j.neuron.2005.05.025  1
2005 Harper SQ, Staber PD, He X, Eliason SL, Martins IH, Mao Q, Yang L, Kotin RM, Paulson HL, Davidson BL. RNA interference improves motor and neuropathological abnormalities in a Huntington's disease mouse model. Proceedings of the National Academy of Sciences of the United States of America. 102: 5820-5. PMID 15811941 DOI: 10.1073/pnas.0501507102  1
2005 Warrick JM, Morabito LM, Bilen J, Gordesky-Gold B, Faust LZ, Paulson HL, Bonini NM. Ataxin-3 suppresses polyglutamine neurodegeneration in Drosophila by a ubiquitin-associated mechanism. Molecular Cell. 18: 37-48. PMID 15808507 DOI: 10.1016/j.molcel.2005.02.030  1
2005 Paulson HL. Autosomal dominant cerebellar ataxia Neurodegenerative Diseases: Neurobiology, Pathogenesis and Therapeutics. 709-718. DOI: 10.1017/CBO9780511544873.048  1
2004 Xia H, Mao Q, Eliason SL, Harper SQ, Martins IH, Orr HT, Paulson HL, Yang L, Kotin RM, Davidson BL. RNAi suppresses polyglutamine-induced neurodegeneration in a model of spinocerebellar ataxia. Nature Medicine. 10: 816-20. PMID 15235598 DOI: 10.1038/nm1076  1
2004 Berke SJ, Schmied FA, Brunt ER, Ellerby LM, Paulson HL. Caspase-mediated proteolysis of the polyglutamine disease protein ataxin-3. Journal of Neurochemistry. 89: 908-18. PMID 15140190 DOI: 10.1111/j.1471-4159.2004.02369.x  1
2004 Gonzalez-Alegre P, Paulson HL. Aberrant cellular behavior of mutant torsinA implicates nuclear envelope dysfunction in DYT1 dystonia. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 24: 2593-601. PMID 15028751 DOI: 10.1523/JNEUROSCI.4461-03.2004  1
2004 Davidson BL, Paulson HL. Molecular medicine for the brain: silencing of disease genes with RNA interference. The Lancet. Neurology. 3: 145-9. PMID 14980529 DOI: 10.1016/S1474-4422(04)00678-7  1
2004 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 Research. 32: 661-8. PMID 14754988 DOI: 10.1093/nar/gkh208  1
2004 Chow MK, Paulson HL, Bottomley SP. Destabilization of a non-pathological variant of ataxin-3 results in fibrillogenesis via a partially folded intermediate: a model for misfolding in polyglutamine disease. Journal of Molecular Biology. 335: 333-41. PMID 14659761 DOI: 10.1016/j.jmb.2003.08.064  1
2004 Chai Y, Berke SS, Cohen RE, Paulson HL. Poly-ubiquitin binding by the polyglutamine disease protein ataxin-3 links its normal function to protein surveillance pathways. The Journal of Biological Chemistry. 279: 3605-11. PMID 14602712 DOI: 10.1074/jbc.M310939200  1
2003 Paulson H. Polyglutamine neurodegeneration: minding your Ps and Qs. Nature Medicine. 9: 825-6. PMID 12835695 DOI: 10.1038/nm0703-825  1
2003 Berke SJ, Paulson HL. Protein aggregation and the ubiquitin proteasome pathway: gaining the UPPer hand on neurodegeneration. Current Opinion in Genetics & Development. 13: 253-61. PMID 12787787 DOI: 10.1016/S0959-437X(03)00053-4  1
2003 Paulson HL, Paulson GW. Genetics of pediatric movement disorders. Seminars in Pediatric Neurology. 10: 88-95. PMID 12785752 DOI: 10.1016/S1071-9091(02)00014-1  1
2003 Gonzalez-Alegre P, Miller VM, Davidson BL, Paulson HL. Toward therapy for DYT1 dystonia: allele-specific silencing of mutant TorsinA. Annals of Neurology. 53: 781-7. PMID 12783425 DOI: 10.1002/ana.10548  1
2003 Miller VM, Xia H, Marrs GL, Gouvion CM, Lee G, Davidson BL, Paulson HL. Allele-specific silencing of dominant disease genes. Proceedings of the National Academy of Sciences of the United States of America. 100: 7195-200. PMID 12782788 DOI: 10.1073/pnas.1231012100  1
2003 Rüb U, Brunt ER, Gierga K, Schultz C, Paulson H, de Vos RA, Braak H. The nucleus raphe interpositus in spinocerebellar ataxia type 3 (Machado-Joseph disease). Journal of Chemical Neuroanatomy. 25: 115-27. PMID 12663059 DOI: 10.1016/S0891-0618(02)00099-6  1
2003 Rüb U, Brunt ER, Del Turco D, de Vos RA, Gierga K, Paulson H, Braak H. Guidelines for the pathoanatomical examination of the lower brain stem in ingestive and swallowing disorders and its application to a dysphagic spinocerebellar ataxia type 3 patient. Neuropathology and Applied Neurobiology. 29: 1-13. PMID 12581335 DOI: 10.1046/j.1365-2990.2003.00437.x  1
2003 Paulson H, Subramony SH. Spinocerebellar Ataxia 3-Machado-Joseph Disease (SCA3) Genetics of Movement Disorders. 57-69. DOI: 10.1016/B978-012566652-7/50007-1  1
2002 Paulson H. Yet another spinocerebellar ataxia--will it ever end? The Lancet. Neurology. 1: 471. PMID 12849326  1
2002 Paulson HL. Diagnostic testing in neurogenetics. Principles, limitations, and ethical considerations. Neurologic Clinics. 20: 627-43, v. PMID 12432824 DOI: 10.1016/S0733-8619(02)00009-9  1
2002 Rüb U, de Vos RA, Brunt ER, Schultz C, Paulson H, Del Tredici K, Braak H. Degeneration of the external cuneate nucleus in spinocerebellar ataxia type 3 (Machado-Joseph disease). Brain Research. 953: 126-34. PMID 12384246 DOI: 10.1016/S0006-8993(02)03278-X  1
2002 Paulson HL, Garbern JY, Hoban TF, Krajewski KM, Lewis RA, Fischbeck KH, Grossman RI, Lenkinski R, Kamholz JA, Shy ME. Transient central nervous system white matter abnormality in X-linked Charcot-Marie-Tooth disease. Annals of Neurology. 52: 429-34. PMID 12325071 DOI: 10.1002/ana.10305  1
2002 Xia H, Mao Q, Paulson HL, Davidson BL. siRNA-mediated gene silencing in vitro and in vivo. Nature Biotechnology. 20: 1006-10. PMID 12244328 DOI: 10.1038/nbt739  1
2002 Paulson HL. Establishing the role of gene-environment interactions in the etiology of type 2 diabetes Endocrinology and Metabolism Clinics of North America. 31: 553-566. PMID 12227119 DOI: 10.1016/S0889-8529(02)00007-5  1
2002 Rüb U, de Vos RA, Schultz C, Brunt ER, Paulson H, Braak H. Spinocerebellar ataxia type 3 (Machado-Joseph disease): severe destruction of the lateral reticular nucleus. Brain : a Journal of Neurology. 125: 2115-24. PMID 12183356  1
2002 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. Proceedings of the National Academy of Sciences of the United States of America. 99: 9310-5. PMID 12084819 DOI: 10.1073/pnas.152101299  1
2002 Paulson H. Yet another spinocerebellar ataxia - Will it ever end? Lancet Neurology. 1: 471. DOI: 10.1016/S1474-4422(02)00234-X  1
2001 Chai Y, Wu L, Griffin JD, Paulson HL. The role of protein composition in specifying nuclear inclusion formation in polyglutamine disease. The Journal of Biological Chemistry. 276: 44889-97. PMID 11572863 DOI: 10.1074/jbc.M106575200  1
2001 Paulson H, Ammache Z. Ataxia and hereditary disorders. Neurologic Clinics. 19: 759-82, viii. PMID 11532653 DOI: 10.1016/S0733-8619(05)70044-X  1
2000 Chan HY, Warrick JM, Gray-Board GL, Paulson HL, Bonini NM. Mechanisms of chaperone suppression of polyglutamine disease: selectivity, synergy and modulation of protein solubility in Drosophila. Human Molecular Genetics. 9: 2811-20. PMID 11092757  1
2000 Paulson HL, Bonini NM, Roth KA. Polyglutamine disease and neuronal cell death. Proceedings of the National Academy of Sciences of the United States of America. 97: 12957-8. PMID 11058149 DOI: 10.1073/pnas.210395797  1
2000 McCampbell A, Taylor JP, Taye AA, Robitschek J, Li M, Walcott J, Merry D, Chai Y, Paulson H, Sobue G, Fischbeck KH. CREB-binding protein sequestration by expanded polyglutamine. Human Molecular Genetics. 9: 2197-202. PMID 10958659  1
2000 Paulson HL. Toward an understanding of polyglutamine neurodegeneration. Brain Pathology (Zurich, Switzerland). 10: 293-9. PMID 10764049  1
2000 Paulson HL, Bonini NM. Spinocerebellar ataxia type 3 Neuroscience News. 3: 87-93.  1
1999 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. Nature Genetics. 23: 425-8. PMID 10581028 DOI: 10.1038/70532  1
1999 Chai Y, Koppenhafer SL, Bonini NM, Paulson HL. Analysis of the role of heat shock protein (Hsp) molecular chaperones in polyglutamine disease. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. 19: 10338-47. PMID 10575031  1
1999 Perez MK, Paulson HL, Pittman RN. Ataxin-3 with an altered conformation that exposes the polyglutamine domain is associated with the nuclear matrix. Human Molecular Genetics. 8: 2377-85. PMID 10556285  1
1999 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. Human Molecular Genetics. 8: 673-82. PMID 10072437  1
1999 Paulson HL. Protein fate in neurodegenerative proteinopathies: polyglutamine diseases join the (mis)fold. American Journal of Human Genetics. 64: 339-45. PMID 9973270 DOI: 10.1086/302269  1
1999 Paulson H. Molecular and pathologic studies of SCA 3/MJD: Toward an understanding of polyglutamine pathogenesis Clinical Neurology. 39: 1282.  1
1998 Erhardt JA, Hynicka W, DiBenedetto A, Shen N, Stone N, Paulson H, Pittman RN. A novel F box protein, NFB42, is highly enriched in neurons and induces growth arrest. The Journal of Biological Chemistry. 273: 35222-7. PMID 9857061 DOI: 10.1074/jbc.273.52.35222  1
1998 Perez MK, Paulson HL, Pendse SJ, Saionz SJ, Bonini NM, Pittman RN. Recruitment and the role of nuclear localization in polyglutamine-mediated aggregation. The Journal of Cell Biology. 143: 1457-70. PMID 9852144 DOI: 10.1083/jcb.143.6.1457  1
1998 Warrick JM, Paulson HL, Gray-Board GL, Bui QT, Fischbeck KH, Pittman RN, Bonini NM. Expanded polyglutamine protein forms nuclear inclusions and causes neural degeneration in Drosophila. Cell. 93: 939-49. PMID 9635424 DOI: 10.1016/S0092-8674(00)81200-3  1
1998 Brooks BP, Merry DE, Paulson HL, Lieberman AP, Kolson DL, Fischbeck KH. A cell culture model for androgen effects in motor neurons. Journal of Neurochemistry. 70: 1054-60. PMID 9489725  1
1997 Paulson HL, Perez MK, Trottier Y, Trojanowski JQ, Subramony SH, Das SS, Vig P, Mandel JL, Fischbeck KH, Pittman RN. Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia type 3. Neuron. 19: 333-44. PMID 9292723 DOI: 10.1016/S0896-6273(00)80943-5  1
1997 Brooks BP, Paulson HL, Merry DE, Salazar-Grueso EF, Brinkmann AO, Wilson EM, Fischbeck KH. Characterization of an expanded glutamine repeat androgen receptor in a neuronal cell culture system. Neurobiology of Disease. 3: 313-23. PMID 9173928 DOI: 10.1006/nbdi.1997.0126  1
1997 Paulson HL, Das SS, Crino PB, Perez MK, Patel SC, Gotsdiner D, Fischbeck KH, Pittman RN. Machado-Joseph disease gene product is a cytoplasmic protein widely expressed in brain. Annals of Neurology. 41: 453-62. PMID 9124802 DOI: 10.1002/ana.410410408  1
1996 Paulson HL, Fischbeck KH. Trinucleotide repeats in neurogenetic disorders. Annual Review of Neuroscience. 19: 79-107. PMID 8833437 DOI: 10.1146/annurev.ne.19.030196.000455  1
1994 La Spada AR, Paulson HL, Fischbeck KH. Trinucleotide repeat expansion in neurological disease. Annals of Neurology. 36: 814-22. PMID 7998766 DOI: 10.1002/ana.410360604  1
1994 Paulson HL, Galetta SL, Grossman M, Alavi A. Hemiachromatopsia of unilateral occipitotemporal infarcts. American Journal of Ophthalmology. 118: 518-23. PMID 7943133  1
1991 Paulson HL, Ross AF, Green WN, Claudio T. Analysis of early events in acetylcholine receptor assembly. The Journal of Cell Biology. 113: 1371-84. PMID 2045417  1
1990 Paulson HL, Claudio T. Temperature-sensitive expression of all-Torpedo and Torpedo-rat hybrid AChR in mammalian muscle cells. The Journal of Cell Biology. 110: 1705-17. PMID 2335568 DOI: 10.1083/jcb.110.5.1705  1
1989 Claudio T, Paulson HL, Green WN, Ross AF, Hartman DS, Hayden D. Fibroblasts transfected with Torpedo acetylcholine receptor beta-, gamma-, and delta-subunit cDNAs express functional receptors when infected with a retroviral alpha recombinant. The Journal of Cell Biology. 108: 2277-90. PMID 2472403  1
1988 Claudio T, Paulson HL, Hartman D, Sine S, Sigworth FJ. Chapter 11 Establishing a Stable Expression System for Studies of Acetylcholine Receptors Current Topics in Membranes and Transport. 33: 219-247. DOI: 10.1016/S0070-2161(08)60901-6  1
1987 Claudio T, Green WN, Hartman DS, Hayden D, Paulson HL, Sigworth FJ, Sine SM, Swedlund A. Genetic reconstitution of functional acetylcholine receptor channels in mouse fibroblasts. Science (New York, N.Y.). 238: 1688-94. PMID 3686008  1
1985 Paulson HL, Byers SW, Dym M. Morphometric analysis of coated pits and vesicles in the proximal and distal caput epididymidis. Biology of Reproduction. 32: 191-202. PMID 2857582  1
1984 Byers SW, Qasba PK, Paulson HL, Dym M. Immunocytochemical localization of alpha-lactalbumin in the male reproductive tract. Biology of Reproduction. 30: 171-8. PMID 6199047  1
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