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

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

88 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
2022 Schuster KH, Zalon AJ, Zhang H, DiFranco DM, Stec NR, Haque Z, Blumenstein KG, Pierce AM, Guan Y, Paulson HL, McLoughlin HS. Impaired oligodendrocyte maturation is an early feature in SCA3 disease pathogenesis. The Journal of Neuroscience : the Official Journal of the Society For Neuroscience. PMID 35042771 DOI: 10.1523/JNEUROSCI.1954-20.2021  0.327
2020 Liu EA, Schultz ML, Mochida C, Chung C, Paulson HL, Lieberman AP. Fbxo2 mediates clearance of damaged lysosomes and modifies neurodegeneration in the Niemann-Pick C brain. Jci Insight. PMID 32931479 DOI: 10.1172/Jci.Insight.136676  0.555
2020 Gerson JE, Safren N, Fischer S, Patel R, Crowley EV, Welday JP, Windle AK, Barmada S, Paulson HL, Sharkey LM. Ubiquilin-2 differentially regulates polyglutamine disease proteins. Human Molecular Genetics. PMID 32681165 DOI: 10.1093/hmg/ddaa152  0.34
2020 Yang CY, Lai RY, Amokrane N, Lin CY, Figueroa KP, Pulst SM, Perlman S, Wilmot G, Gomez CM, Schmahmann JD, Paulson H, Shakkottai VG, Rosenthal LS, Ying SH, Zesiewicz T, et al. Dysphagia in spinocerebellar ataxias type 1, 2, 3 and 6. Journal of the Neurological Sciences. 415: 116878. PMID 32454319 DOI: 10.1016/J.Jns.2020.116878  0.318
2020 Gan SR, Figueroa KP, Xu HL, Perlman S, Wilmot G, Gomez CM, Schmahmann J, Paulson H, Shakkottai VG, Ying SH, Zesiewicz T, Bushara K, Geschwind MD, Xia G, Subramony SH, et al. The impact of ethnicity on the clinical presentations of spinocerebellar ataxia type 3. Parkinsonism & Related Disorders. 72: 37-43. PMID 32105964 DOI: 10.1016/J.Parkreldis.2020.02.004  0.385
2019 Schultz ML, Fawaz MV, Azaria RD, Hollon TC, Liu EA, Kunkel TJ, Halseth TA, Krus KL, Ming R, Morin EE, McLoughlin HS, Bushart DD, Paulson HL, Shakkottai VG, Orringer DA, et al. Synthetic high-density lipoprotein nanoparticles for the treatment of Niemann-Pick diseases. Bmc Medicine. 17: 200. PMID 31711490 DOI: 10.1186/S12916-019-1423-5  0.503
2019 Paulson H. Repeat Expansions in Leukoencephalopathy. Annals of Neurology. PMID 31600824 DOI: 10.1002/Ana.25613  0.364
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  0.515
2019 Johnson SL, Blount JR, Libohova K, Ranxhi B, Paulson HL, Tsou WL, Todi SV. Differential toxicity of ataxin-3 isoforms in Drosophila models of Spinocerebellar Ataxia Type 3. Neurobiology of Disease. 104535. PMID 31310802 DOI: 10.1016/j.nbd.2019.104535  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  0.523
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  0.357
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  0.54
2018 Paulson H. Repeat expansion diseases. Handbook of Clinical Neurology. 147: 105-123. PMID 29325606 DOI: 10.1016/B978-0-444-63233-3.00009-9  0.422
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  0.312
2017 Ramani B, Panwar B, Moore LR, Wang B, Huang R, Guan Y, Paulson HL. Comparison of spinocerebellar ataxia type 3 mouse models identifies early gain-of-function, cell-autonomous transcriptional changes in oligodendrocytes. Human Molecular Genetics. 26: 3362-3374. PMID 28854700 DOI: 10.1093/Hmg/Ddx224  0.331
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  0.541
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  0.31
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  0.432
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  0.319
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  0.735
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  0.302
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  0.359
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  0.542
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  0.535
2014 Atkin G, Paulson H. Ubiquitin pathways in neurodegenerative disease. Frontiers in Molecular Neuroscience. 7: 63. PMID 25071440 DOI: 10.3389/Fnmol.2014.00063  0.381
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  0.344
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  0.588
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  0.464
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  0.308
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  0.399
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  0.775
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  0.395
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  0.308
2013 Todd P, Oh S, Krans A, He F, Sellier C, Frazer M, Renoux A, Chen K, Scaglione K, Basrur V, Elenitoba-Johnson K, Vonsattel J, Louis E, Sutton M, Taylor J, ... ... Paulson H, et al. CGG Repeat-Associated Translation Mediates Neurodegeneration in Fragile X Tremor Ataxia Syndrome Neuron. 79: 402. DOI: 10.1016/j.neuron.2013.07.008  0.614
2013 Rüb U, Hentschel M, Brunt E, Heinsen H, Seidel K, Stratmann K, Paulson H, Vonsattel J-, Dunnen Wd, Korf H-. Degeneration of the cerebellum and brainstem in Huntington's disease (HD) Journal of the Neurological Sciences. 333. DOI: 10.1016/J.Jns.2013.07.471  0.357
2013 Blair L, Nordhues B, Hill S, Scaglione KM, O'Leary J, Breydo L, Bo Z, Li P, Wang L, Cotman C, Paulson H, Muschol M, Uversky V, Klengel T, Binder E, et al. The Hsp90 co-chaperone FKBP51 produces neurotoxic tau oligomers: Implication for aging and Alzheimer's disease Alzheimers & Dementia. 9: 173. DOI: 10.1016/J.Jalz.2013.05.279  0.339
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  0.329
2012 Todd PK, Paulson HL. Drug discovery: Kill the messenger where it lives. Nature. 488: 36-8. PMID 22859197 DOI: 10.1038/488036a  0.333
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  0.539
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  0.315
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  0.414
2012 Figueroa K, Gomez C, Paulson H, Perlman S, Schmahmann J, Subramony S, Wilmot G, Zesiewicz T, Ashizawa T, Pulst S. Prior Molecular Diagnostic Accuracy and Age of Disease Onset Variation in the CRC-SCA, a Multicenter Study of Spinocerebellar Ataxias (S12.003) Neurology. 78: S12.003-S12.003. DOI: 10.1212/Wnl.78.1_Meetingabstracts.S12.003  0.314
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  0.365
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  0.648
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  0.31
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  0.354
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  0.497
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  0.577
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  0.327
2009 Sabine C, Trembath K, Churchyard A, Horton Z, Tippett L, Hogg V, Roxburgh R, Velakoulis D, Collins V, Delatycki M, Aylward E, Nopoulos P, Johnson H, Juhl A, Magnotta V, ... ... Paulson H, et al. Second Annual Huntington Disease Clinical Research Symposium Neurotherapeutics. 6: 202-212. DOI: 10.1016/J.Nurt.2008.09.004  0.301
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  0.347
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  0.332
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  0.304
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  0.35
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  0.344
2006 McKay EC, Beck JS, Winn M, Paulson HL, Lieberman AP, Counts SE. P3-208: THE OXYTOCIN RECEPTOR AND VASCULAR COGNITIVE IMPAIRMENT: POTENTIAL AS A NOVEL THERAPEUTIC TARGET Alzheimer's & Dementia. 14: P1148-P1149. DOI: 10.1016/j.jalz.2018.06.1567  0.471
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  0.324
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  0.317
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  0.308
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  0.315
2003 Paulson H. Polyglutamine neurodegeneration: minding your Ps and Qs. Nature Medicine. 9: 825-6. PMID 12835695 DOI: 10.1038/Nm0703-825  0.318
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  0.329
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  0.36
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  0.484
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 DOI: 10.1093/Brain/Awf208  0.361
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  0.345
2001 Paulson H. Death by polyglutamine: expanding our knowledge Trends in Neurosciences. 24: 547. DOI: 10.1016/S0166-2236(00)01900-7  0.386
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 DOI: 10.1093/Hmg/9.19.2811  0.304
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  0.339
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 DOI: 10.1093/Hmg/9.14.2197  0.777
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  0.316
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  0.521
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  0.344
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  0.713
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  0.525
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  0.682
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 DOI: 10.1046/J.1471-4159.1998.70031054.X  0.638
1998 Opal P, Paulson H. Genetic Instabilities and Hereditary Neurological Diseases The American Journal of Human Genetics. 63: 1921. DOI: 10.1086/302134  0.385
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  0.664
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  0.498
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  0.672
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  0.561
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  0.689
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 DOI: 10.1083/Jcb.113.6.1371  0.626
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  0.605
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 DOI: 10.1083/Jcb.108.6.2277  0.627
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  0.626
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 DOI: 10.1126/Science.3686008  0.642
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