HenryLaurisPaulsonMD

PubMed

• Caregiver Experiences and Burden in Moderate-Advanced Dementia With Lewy Bodies.
  Yunfeng Dai, Kaitlin Sovich, Brian LaBarre, Julie A Fields, Angela M Lunde, Leah K Forsberg, Carol A Manning, Angela S Taylor, Zhigang Li, Melissa J Armstrong, Henry L...> ;Neurology. Clinical Practice. 2024 Jun 1
• 1 citations
  Pathogenic mutations in UBQLN2 exhibit diverse aggregation propensity and neurotoxicity.
  Nathaniel Safren, Thuy P Dao, Harihar Milaganur Mohan, Camellia Huang, Bryce Trotter, Carlos A Castañeda, Henry Paulson, Sami Barmada, Lisa M Sharkey> ;Scientific Reports. 2024 Mar 13
• Fatigue Impacts Quality of Life in People with Spinocerebellar Ataxias.
  Ruo-Yah Lai, Christian Rummey, Christian J Amlang, Chi-Ying R Lin, Tiffany X Chen, Susan Perlman, George Wilmot, Christopher M Gomez, Jeremy D Schmahmann, Henry Paulso...> ;Movement Disorders Clinical Practice. 2024 Feb 29
• Patient- and proxy-reported quality of life in advanced dementia with Lewy bodies.
  Melissa J Armstrong, Brian LaBarre, Kaitlin Sovich, Susan M Maixner, Henry L Paulson, Carol Manning, Julie A Fields, Angela Lunde, Leah Forsberg, Bradley F Boeve, Jame...> ;Alzheimer's & Dementia. 2024 Feb 23
• Lessons Learned from Approval of Aducanumab for Alzheimer's Disease.
  Heidebrink, J., Paulson, H.> ;Annual Review of Medicine. 2024 Jan 29
• The Cerebellar Cognitive Affective/Schmahmann Syndrome Scale in Spinocerebellar Ataxias.
  Selvadurai, L., Perlman, S., Ashizawa, T., Wilmot, G., Onyike, C., Rosenthal, L., Shakkottai, V., Paulson, H., Subramony, S., Bushara, K., Kuo, S., Dietiker, C., Gesch...> ;Cerebellum. 2024 Jan 2
• 1 citations
  Wild-type and pathogenic forms of ubiquilin 2 differentially modulate components of the autophagy-lysosome pathways.
  Akiko Idera, Lisa M Sharkey, Yuki Kurauchi, Keiichi Kadoyama, Henry L Paulson, Hiroshi Katsuki, Takahiro Seki> ;Journal of Pharmacological Sciences. 2023 Jul 1
• Evaluation of the Uniform Data Set version 3 teleneuropsychological measures.
  Theresa F Gierzynski, Allyson Gregoire, Jonathan M Reader, Rebecca Pantis, Stephen Campbell, Arijit Bhaumik, Annalise Rahman-Filipiak, Judith Heidebrink, Bruno Giordan...> ;Journal of the International Neuropsychological Society. 2024 Feb 1
• 2 citations
  Research Priorities of Individuals and Caregivers With Lewy Body Dementia: A Web-based Survey.
  Goldman, J. G., Goldman, J. G., Goldman, J. G., Samantha K Holden, Noheli Bedenfield, Angela S Taylor, Ece Bayram, Chris Schwilk, Jori Fleisher, John Duda, Holly Shill...> ;Alzheimer Disease and Associated Disorders. 2023 Feb 23
• 2 citations
  Baseline Clinical and Blood Biomarkers in Patients With Preataxic and Early-Stage Disease Spinocerebellar Ataxia 1 and 3.
  Sophie Tezenas du Montcel, Emilien Petit, Titilayo Olubajo, Jennifer Faber, Pauline Lallemant-Dudek, Khalaf Bushara, Susan Perlman, Sub H Subramony, David Morgan, Bria...> ;Neurology. 2023 Apr 25
• Ubiquilin-2 regulates pathological alpha-synuclein.
  Stephanie S Sandoval-Pistorius, Julia E Gerson, Nyjerus Liggans, Jaimie H Ryou, Kulin Oak, Xingli Li, Keyshla Y Negron-Rios, Svetlana Fischer, Henry Barsh, Emily V Cro...> ;Scientific Reports. 2023 Jan 6
• 4 citations
  Regional and age-dependent changes in ubiquitination in cellular and mouse models of spinocerebellar ataxia type 3.
  Haiyang Luo, Sokol V Todi, Henry L Paulson, Maria do Carmo Costa> ;Frontiers in Molecular Neuroscience. 2023 Jan 1
• Quantitative flow cytometric selection of tau conformational nanobodies specific for pathological aggregates.
  Jennifer M Zupancic, Matthew D Smith, Hanna Trzeciakiewicz, Mary E Skinner, Sean P Ferris, Emily K Makowski, Michael J Lucas, Nikki McArthur, Ravi S Kane, Henry L Paul...> ;Frontiers in Immunology. 2023 Jan 1
• Upregulation of the ESCRT pathway and multivesicular bodies accelerates degradation of proteins associated with neurodegeneration.
  Ron Benyair, Sai Srinivas Panapakkam Giridharan, Pilar Rivero-Ríos, Junya Hasegawa, Emily Bristow, Eeva-Liisa Eskelinen, Merav D Shmueli, Vered Fishbain-Yoskovitz, Yif...> ;Autophagy Reports. 2023 Jan 1
• 11 citations
  Clinically Meaningful Magnetic Resonance Endpoints Sensitive to Preataxic Spinocerebellar Ataxia Types 1 and 3.
  Jayashree Chandrasekaran, Emilien Petit, Young Woo Park, Sophie Tezenas du Montcel, James M Joers, Dinesh K Deelchand, Michal Považan, Guita Banan, Romain Valabregue, ...> ;Annals of Neurology. 2023 Apr 1
• 1 citations
  Sleep Alterations in a Mouse Model of Spinocerebellar Ataxia Type 3.
  Maria-Efstratia Tsimpanouli, Anjesh Ghimire, Anna J Barget, Ridge Weston, Henry L Paulson, Maria do Carmo Costa, Brendon O Watson> ;Cells. 2022 Oct 5
• 4 citations
  The S-Factor, a New Measure of Disease Severity in Spinocerebellar Ataxia: Findings and Implications.
  Louisa P Selvadurai, Susan L Perlman, George R Wilmot, Sub H Subramony, Christopher M Gomez, Tetsuo Ashizawa, Henry L Paulson, Chiadi U Onyike, Liana S Rosenthal, Hari...> ;Cerebellum. 2023 Oct 1
• Derivation of spinocerebellar ataxia type 3 human embryonic stem cell line UMICHe001-A/UM134-1.
  Lauren R Moore, Laura Keller, Henry L Paulson, Gary D Smith> ;Stem Cell Research. 2022 Oct 1
• 10 citations
  GRASP55 regulates the unconventional secretion and aggregation of mutant huntingtin.
  Erpan Ahat, Sarah Bui, Jianchao Zhang, Felipe da Veiga Leprevost, Lisa Sharkey, Whitney Reid, Alexey I Nesvizhskii, Henry L Paulson, Yanzhuang Wang> ;The Journal of Biological Chemistry. 2022 Aug 1
• 10 citations
  Positive Airway Pressure and Cognitive Disorders in Adults With Obstructive Sleep Apnea: A Systematic Review of the Literature.
  Monica Moon Shieu, Carol Shannon, Ronald David Chervin, Alan Conceicao, Henry Lauris Paulson, Galit Levi Dunietz, Zaheed, A., Braley, T.> ;Neurology. 2022 Jul 25
• 14 citations
  The association between obstructive sleep apnea risk and cognitive disorders: a population-based study.
  Monica M Shieu, Galit Levi Dunietz, Henry L Paulson, Ronald D Chervin, Tiffany J Braley> ;Journal of Clinical Sleep Medicine. 2022 Apr 1
• 1 citations
  RTL8 promotes nuclear localization of UBQLN2 to subnuclear compartments associated with protein quality control.
  Harihar Milaganur Mohan, Hanna Trzeciakiewicz, Amit Pithadia, Emily V Crowley, Regina Pacitto, Nathaniel Safren, Bryce Trotter, Chengxin Zhang, Xiaogen Zhou, Yang Zhan...> ;Cellular and Molecular Life Sciences. 2022 Mar 5
• 4 citations
  Disrupting the Balance of Protein Quality Control Protein UBQLN2 Accelerates Tau Proteinopathy.
  Julia E Gerson, Stephanie Sandoval-Pistorius, Jacqueline P Welday, Aleija Rodriguez, Jordan D Gregory, Nyjerus Liggans, Kylie Schache, Xingli Li, Hanna Trzeciakiewicz,...> ;The Journal of Neuroscience. 2022 Mar 2
• 18 citations
  Impaired Oligodendrocyte Maturation Is an Early Feature in SCA3 Disease Pathogenesis.
  Kristen H Schuster, Annie J Zalon, Hongjiu Zhang, Danielle M DiFranco, Nicholas R Stec, Zaid Haque, Kate G Blumenstein, Amanda M Pierce, Yuanfang Guan, Henry L Paulson...> ;The Journal of Neuroscience. 2022 Feb 23
• 2 citations
  Protocol for an observational cohort study identifying factors predicting accurately end of life in dementia with Lewy bodies and promoting quality end-of-life experie...
  Melissa J. Armstrong, Henry L. Paulson, Susan M. Maixner, Julie A. Fields, Angela Lunde, Bradley F. Boeve, Carol A. Manning, James E. Galvin, Angela Taylor, Zhigang Li> ;BMJ Open. 2021 May 26
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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
• 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
• 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
• 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
• 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
• 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
• 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
• 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
• 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
• 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
• Ubiquitin pathways in neurodegenerative disease  
  Atkin G, Paulson H, Front Mol Neurosci, 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
• 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
• New hope for therapy in neurodegenerative diseases?  
  do Carmo Costa M, Paulson HL, Cell Res, 1/1/2013
• 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
• 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
• 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
• 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
• 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
• Handb Clin Neurol  
  Machado-Joseph disease/spinocerebellar ataxia type 3, Handb Clin Neurol, 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
• 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
• 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
• Movement Disorders: Neurologic Principles and Practice 
  Chapter: Molecular Genetics of the Ataxias 
  Paulson HLEditors: Watts & Koller.McGraw-Hill Companies, Inc.Edition 3rd,2009
• 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
• Handbook of Clinical Neurology 
  Chapter: Machado-Joseph Disease/Spinocerebellar Ataxia Type 3 
  Paulson HLEditors: Aminoff, Boller & Swaab.Elsevier BV2008
• Molecular Neurology 
  Chapter: Polyglutamine Disorders including Huntington's Disease 
  Page 257 - 275Todi S, Williams A, Paulson HEditors: Waxman S.Academic Press2007
• 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
• 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
• 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
• Neurodegenerative Diseases: Neurobiology, Pathogenesis and Therapeutics 
  Chapter: Autosomal Dominant Ataxia 
  Page 709 - 718Paulson HLEditors: Beal F.Cambridge University Press2005
• 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
• 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
• 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
• 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
• 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
• Molecular Mechanisms of Neurological Diseases 
  Chapter: Pathophysiological mechanisms in SCA3 
  Page 285 - 306Opal P, Paulson HEditors: Chesselet M-F.Humana Press, Totowa, NJ2000
• Trinucleotide Repeat Diseases 
  Chapter: Spinocerebellar ataxia type 3/Machado-Joseph disease 
  Paulson HLEditors: Rubinstein DC, Hayden MR,.Bios Scientific Publishers Ltd, Oxford1998
• 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
• Current Diagnosis 9 
  Chapter: The Spinocerebellar Degenerations 
  Paulson HL, Hurtig HIEditors: Conn, Borer, Snyder.WB Saunders Co, Philadelphia, PA1997
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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
• 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
• 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
• Genetics of Dementia 
  La Jolla, CA - 1/1/2012
• Alzheimer's Disease and Related Dementias 
  1/1/2012
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