HenryLaurisPaulsonMD

PubMed

• 132 citations
  Transethnic genome-wide scan identifies novel Alzheimer's disease loci
  Gyungah Jun, Jaeyoon Chung, Jesse Mez, Robert Barber, Gary W. Beecham
  Alzheimer's & Dementia. 2017-07-01
• 194 citations
  A novel Alzheimer disease locus located near the gene encoding tau protein
  Gyungah Jun, Carla A. Ibrahim-Verbaas, Maria Vronskaya, J-C Lambert, Jaeyoon Chung
  Molecular Psychiatry. 2016-01-01
• 514 citations
  RNA interference improves motor and neuropathological abnormalities in a Huntington's disease mouse model.
  Scott Q. Harper, Patrick D. Staber, Xiaohua He, Steven Eliason, Inês Martins
  Proceedings of the National Academy of Sciences of the United States of America. 2005-04-19

Journal Articles

• Mutant UBQLN2 Promotes Toxicity by Modulating Intrinsic Self-Assembly  
  Sami J Barmada, Henry L Paulson, Ronak Patel, Proceedings of the National Academy of Sciences
• Allosteric regulation of deubiquitylase activity through ubiquitination  
  Faggiano S, Menon RP, Kelly GP, Todi SV, Scaglione KM, Konarev PV, Svergun DI, Paulson HL, Pastore A, Front Mol Biosci, 1/1/2015
• Vascular risk factors and clinical progression in spinocerebellar ataxias  
  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, ubram..., Tremor Other Hyperkinet Mov, 1/1/2015
• Intrinsic disorder drives N-terminal ubiquitination by Ube2w  
  Vittal V, Shi L, Wenzel DM, Scaglione KM, Duncan ED, Basrur V, Elenitoba-Johnson KS, Baker D, Paulson HL, Brzovic PS, Klevit RE, Nat Chem Biol, 1/1/2015
• A knock-in mouse model of Spinocerebellar ataxia type 3 exhibits prominent aggregate pathology and aberrant splicing of the disease gene transcript  
  Ramani B, Harris GM, Huang R, Seki T, Murphy GG, Costa MD, Fischer S, Saunders TL, Xia G, McEachin RC, Henry L. Paulson HL, Hum Mol Genet, 1/1/2015
• Dominant negative effect of polyglutamine expansion perturbs normal function of ataxin-3 in neuronal cells  
  Neves-Carvalho A, Logarinho E, Freitas A, Duarte-Silva S, Costa MD, Silva-Fernandes A, Martins M, Serra SC, Lopes AT, Paulson HL, Heutink P, Relvas JB, Maciel P, Hum Mol Genet, 1/1/2015
• Huntington's disease (HD): degeneration of select nuclei, widespread occurrence of neuronal nuclear and axonal inclusions on the brainstem  
  Rub 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, Brain Pathology, 1/1/2014
• Differential Effects of Delayed Aging on Phenotype and Striatal Pathology in a Murine Model of Huntington Disease  
  Tallaksen-Greene S, Sadagurski M, Zeng L, Mauch R, Perkins M, Banduseela V, Lieberman A, Miller R, Paulson H, Albin R, J. Neuroscience, 1/1/2014
• Ubiquitin pathways in neurodegenerative disease  
  Atkin G, Paulson H, Front Mol Neurosci, 1/1/2014
• Alterations in cerebellar physiology are associated with a stiff-legged gait in Atcay (ji-hes) mice  
  Luna-Cancalon K, Sikora KM, Pappas SS, Singh V, Wulff H, Paulson HL, Burmeister M, Shakkottai VG, Neurobiology of Disease, 1/1/2014
• F-box only protein 2 (Fbxo2) Regulates Amyloid Precursor Protein Levels and Processing  
  Atkin G, Hunt J, Minakawa E, Sharkey L, Tipper N, Tennant W, Paulson HL, J Biol Chem, 1/1/2014
• TMEM106B is a genetic modifier of frontotemporal lobar degeneration with C9orf72 hexanucleotide repeat expansions  
  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,..., Acta Neuropathol, 1/1/2014
• Silencing mutant ATXN3 expression resolve molecular phenotypes in SCA3 transgenic mice  
  Rodriguez-Lebron E, do Carmo Costa M, Molina-Luna K, Peron TM, Fischer S, Boudreau RL, Davidson BL, Paulson HL, Mol Ther, 1/1/2013
• New hope for therapy in neurodegenerative diseases?  
  do Carmo Costa M, Paulson HL, Cell Res, 1/1/2013
• Mutations in SLC20A2 are a major cause of familial idiopathic basal ganglia calcification  
  Neurogenetics, 1/1/2013
• Clinical features, neurogenetics and neuropathology of the polyglutamine spinocerebellar ataxias type 1, 2, 3, 6 and 7  
  Rub U, Schols L, Paulson H, Auburger G, Kermer P, Jen JC, Seidel K, Korf HW, Deller T, Prog Neurobiol, 1/1/2013
• CGG Repeat Associated Translation Mediates Neurodegeneration in Fragile X Tremor Ataxia Syndrome  
  Todd PK, Oh SY Krans A, He F, Sellier C, Frazer M, Renoux AJ, Chen K-C, Scaglione KM, Basrur V, Elenitoba-Johnson K, Vonsattel JP, Louis ED, Sutton MA, Taylor JP, Mill..., Neuron, 1/1/2013
• JosD1, a membrane-targeted deubiquitinating enzyme, is activated by ubiquitination and regulates membrane dynamics, cell motility and endocytosis  
  Seki T, Gong L, Williams AJ, Sakai N, Todi SV, Paulson HL, J Biol Chem, 1/1/2013
• The Ubiquitin-conjugating Enzyme (E2) Ube2w Ubiquitinates the N Terminus of Substrates  
  Scaglione KM, Basrur V, Ashraf NS, Konen JR, Elenitoba-Johnson KS, Todi SV, Paulson HL, J Biol Chem, 1/1/2013
• The E3 Ubiquitin Ligase CHIP and the Molecular Chaperone Hsc70 Form a Dynamic, Tethered Complex Biochemistry  
  Smith MC, Scaglione KM, Assimon VA, Patury S, Thompson AD, Dickey CA, Southworth DR, Paulson HL, Gestwicki JE, Zuiderweg ER, 1/1/2013
• Clinical characteristics of patients with spinocerebellar ataxias 1, 2, 3 and 6 in the US; a prospective observational study  
  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, Maz..., Orphanet J Rare Dis, 1/1/2013
• Enzymatic production of mono-ubiquitinated proteins for structural studies: The example of the Josephin domain of ataxin-3 FEBS  
  Faggiano S, Menon RP, Kelly GP, McCormick J, Todi SV, Scaglione KM, Paulson HL, Pastore A, FEBS Open Bio, 1/1/2013
• Accelerated neurodegeneration through chaperone-mediated oligomerization of tau  
  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, Bin..., J Clin Invest, 1/1/2013
• Toward RNAi Therapy for the Polyglutamine Disease Machado-Joseph Disease  
  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, Mol Ther, 1/1/2013
• Drug discovery: Kill the messenger where it lives  
  Todd PK, Paulson HL, Nature, 1/1/2012
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Books/Book Chapters

• Atypical and Non-AD Dementias 
  Chapter: Repeat Expansion Diseases and Dementia 
  Dayalu P, Albin RL, Paulson HLEditors: Michael Geschwind and Caroline Racine.Blackwell Publishing Limited2013
• Handbook of Clinical Neurology, Ataxic Disorders 
  Chapter: Machado-Joseph disease/Spinocerebellar ataxia type 3 
  Paulson HLEditors: Aminoff, Boller and Swaab.Elsevier Pvt. Ltd, IndiaEdition 3rd,2011
• Neurobiology of Huntington's Disease: Applications to Drug Discovery 
  Chapter: Huntington's Disease: Clinical Features and Routes to Therapy 
  Paulson HL, Albin RLEditors: Lo DC, Hughes RE.CRC Press2011
• Movement Disorders: Neurologic Principles and Practice 
  Chapter: Molecular Genetics of the Ataxias 
  Paulson HLEditors: Watts & Koller.McGraw-Hill Companies, Inc.Edition 3rd,2009
• Handbook of Clinical Neurology 
  Chapter: Machado-Joseph Disease/Spinocerebellar Ataxia Type 3 
  Paulson HLEditors: Aminoff, Boller & Swaab.Elsevier BV2008
• Drug Discovery and Development in Huntington Disease. Frontiers in Neuroscience Series 
  Chapter: Huntington's Disease: Clinical Features and Routes to Therapy 
  Paulson HL, Albin RLEditors: Donald C. Lo and Robert E. Hughes.CRC Press, Boca Raton, FL2008
• Protein Misfolding, Aggregation and Conformational Diseases: II. Molecular Mechanisms of Conformational Diseases 
  Chapter: Mechanistic insights into the polyglutamine ataxias 
  Miller VM, Paulson HLEditors: Uversky VN, Fink AL.Kluwer Academic/Plenum Publishers, New York, NY2007
• Spinocerebellar Degenerations: The Ataxias and Spastic Paraplegias 
  Chapter: Cell and animal models of polyglutamine ataxia 
  Page 147 - 171Pulst S, Paulson HEditors: Brice A, Pulst SM.Elsevier, Philadelphia, PA2007
• Molecular Neurology 
  Chapter: Polyglutamine Disorders including Huntington's Disease 
  Page 257 - 275Todi S, Williams A, Paulson HEditors: Waxman S.Academic Press2007
• The Molecular and Genetic Basis of Neurologic and Psychiatric Disease 
  Rosenberg RN, Prusiner SB, DiMauro S, Barchi RL, Nestler EJ, Paulson HL, Ptacek LButterworth/Heinemann, Philadelphia, PA2006
• Genetic Instabilities and Neurological Diseases 
  Chapter: Machado Joseph Disease/Spinocerebellar Ataxia Type 3 
  Page 363 - 377Paulson HLEditors: Wells R, Ashizawa T.Elsevier/Academic Press, San Diego, CAEdition 2nd,2005
• Vectors and RNA Interference for Neuroscience Applications 
  Chapter: RNAi for Disease: Allele-specific Silencing 
  Page 39 - 46Paulson HLEditors: Davidson BL.Society for Neuroscience, Washington, DC2005
• Neurodegenerative Diseases: Neurobiology, Pathogenesis and Therapeutics 
  Chapter: Autosomal Dominant Ataxia 
  Page 709 - 718Paulson HLEditors: Beal F.Cambridge University Press2005
• Genetics of Movement Disorders 
  Chapter: Spinocerebellar ataxia type 3/ Machado-Joseph disease 
  Page 57 - 70Paulson H, Subramony SEditors: Pulst S.Academic Press/Elsevier Science, USA2003
• Molecular and Genetic Basis of Neurological Disease 
  Chapter: The Inherited Ataxias 
  Page 369 - 382Rosenberg R, Paulson HEditors: Rosenberg, Prusiner, DiMauro, Barchi, Nestler.Butterworth/Heineman, Philadelphia, PAEdition 3rd,2003
• Molecular Mechanisms of Neurological Diseases 
  Chapter: Pathophysiological mechanisms in SCA3 
  Page 285 - 306Opal P, Paulson HEditors: Chesselet M-F.Humana Press, Totowa, NJ2000
• Handbook of Ataxia Disorders 
  Chapter: Spinocerebellar Ataxia type 3/Machado-Joseph disease 
  Schols L, Paulson H, Riess OEditors: Kockgether T.Marcel Dekker, CRC Press, New York, NY2000
• Trinucleotide Repeat Diseases 
  Chapter: Spinocerebellar ataxia type 3/Machado-Joseph disease 
  Paulson HLEditors: Rubinstein DC, Hayden MR,.Bios Scientific Publishers Ltd, Oxford1998
• Current Diagnosis 9 
  Chapter: The Spinocerebellar Degenerations 
  Paulson HL, Hurtig HIEditors: Conn, Borer, Snyder.WB Saunders Co, Philadelphia, PA1997
• Movement Disorders: Neurologic Principles and Practice 
  Chapter: Clinical Manifestations of Parkinson's Disease 
  Paulson HL, Stern MBEditors: Watts RL, Koller WC.McGraw-Hill Publishers, New York, NY1997
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Lectures

• Polyglutamine and other repeat expansion diseases: Clinical presentation 
  Braga, Portugal - 1/1/2015
• Polyglutamine diseases: mechanisms revisited 
  Braga, Portugal - 1/1/2015
• Polyglutamine diseases: Innovative therapies 
  Braga, Portugal - 1/1/2015
• Developing therapies for polyglutamine diseases 
  Tokyo, Japan - 1/1/2015
• Ubiquitin pathways in neurodegenerative diseases 
  Niigata, Japan - 1/1/2015
• Genetics of Dementias 
  Philadelphia, PA - 1/1/2014
• Protein Quality Control 
  1/1/2014
• Protein Misfolding Diseases 
  Ann Arbor, MI, 2009 - 1/1/2014
• Neurobiology of Parkinson's Disease 
  1/1/2014
• New insights into protein quality control in age-related neurodegeneration 
  New York, NY - 1/1/2014
• Gene Silencing for Other Neurodegenerative Diseases 
  Stockholm, Sweden - 1/1/2014
• Ubiquitin pathways in neurodegenerative disease: an update 
  Durham, NC - 1/1/2014
• Update on Alzheimer's disease and related dementias 
  Ann Arbor, MI, 2012; Southfield, MI, 2013 - 1/1/2013
• 3FC.003: Genetics in Neurology 
  San Diego, CA - 1/1/2013
• Toward understanding and treating polyglutamine neurodegeneration 
  Multiple locations, 2011, 2012, 2013, 2015 - 1/1/2013
• Spinocerebellar ataxia: Research progress and developing treatment 
  Tokyo, Japan - 1/1/2013
• Advances in Research on the Different "Faces" of Dementia 
  Detroit, MI - 1/1/2013
• Toward Understanding and Treating Machado Joseph Disease (Spinocerebellar Ataxia Type 3) 
  Framingham, MA - 1/1/2013
• Cerebellar ataxias: future directions in genetics and therapeutics 
  Sao Paulo, Brazil - 1/1/2013
• Protein Quality Control in Age-Related Neurodegeneration 
  Ann Arbor, MI - 1/1/2013
• Ataxia: the Michigan Experience 
  Detroit, MI - 1/1/2013
• Toward Therapy for polyglutamine ataxias 
  Tokyo, Japan - 1/1/2013
• Disobeying the rules: studies of neurodegenerative diseases leading to new insights on translation and ubiquitination 
  Ypsilanti Eagle Crest Conference Center - 1/1/2013
• Alzheimer's Disease and Related Dementias 
  1/1/2012
• Fraud, Fabrication, and Plagiarism 
  1/1/2012
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