Henry L Paulson

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Henry L Paulson

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The ubiquitin conjugating enzyme Ube2W regulates solubility of the Huntington's disease protein, huntingtin.

Neurobiol Dis 2018 Jan 3;109(Pt A):127-136. Epub 2017 Oct 3.

Department of Neurology, University of Michigan, Ann Arbor, MI 48109, USA; Neuroscience Program, University of Michigan, Ann Arbor, MI 48109, USA. Electronic address:

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January 2018

Preface.

Handb Clin Neurol 2018 ;147:ix-x

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January 2018

Preface.

Handb Clin Neurol 2018 ;148:ix-x

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January 2018

Polyglutamine spinocerebellar ataxias - from genes to potential treatments.

Nat Rev Neurosci 2017 10 17;18(10):613-626. Epub 2017 Aug 17.

Department of Laboratory Medicine and Pathology, University of Minnesota, Minneapolis, Minnesota, 55455, USA.

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October 2017

Rare coding variants in PLCG2, ABI3, and TREM2 implicate microglial-mediated innate immunity in Alzheimer's disease.

Authors:
Rebecca Sims Sven J van der Lee Adam C Naj Céline Bellenguez Nandini Badarinarayan Johanna Jakobsdottir Brian W Kunkle Anne Boland Rachel Raybould Joshua C Bis Eden R Martin Benjamin Grenier-Boley Stefanie Heilmann-Heimbach Vincent Chouraki Amanda B Kuzma Kristel Sleegers Maria Vronskaya Agustin Ruiz Robert R Graham Robert Olaso Per Hoffmann Megan L Grove Badri N Vardarajan Mikko Hiltunen Markus M Nöthen Charles C White Kara L Hamilton-Nelson Jacques Epelbaum Wolfgang Maier Seung-Hoan Choi Gary W Beecham Cécile Dulary Stefan Herms Albert V Smith Cory C Funk Céline Derbois Andreas J Forstner Shahzad Ahmad Hongdong Li Delphine Bacq Denise Harold Claudia L Satizabal Otto Valladares Alessio Squassina Rhodri Thomas Jennifer A Brody Liming Qu Pascual Sánchez-Juan Taniesha Morgan Frank J Wolters Yi Zhao Florentino Sanchez Garcia Nicola Denning Myriam Fornage John Malamon Maria Candida Deniz Naranjo Elisa Majounie Thomas H Mosley Beth Dombroski David Wallon Michelle K Lupton Josée Dupuis Patrice Whitehead Laura Fratiglioni Christopher Medway Xueqiu Jian Shubhabrata Mukherjee Lina Keller Kristelle Brown Honghuang Lin Laura B Cantwell Francesco Panza Bernadette McGuinness Sonia Moreno-Grau Jeremy D Burgess Vincenzo Solfrizzi Petra Proitsi Hieab H Adams Mariet Allen Davide Seripa Pau Pastor L Adrienne Cupples Nathan D Price Didier Hannequin Ana Frank-García Daniel Levy Paramita Chakrabarty Paolo Caffarra Ina Giegling Alexa S Beiser Vilmantas Giedraitis Harald Hampel Melissa E Garcia Xue Wang Lars Lannfelt Patrizia Mecocci Gudny Eiriksdottir Paul K Crane Florence Pasquier Virginia Boccardi Isabel Henández Robert C Barber Martin Scherer Lluis Tarraga Perrie M Adams Markus Leber Yuning Chen Marilyn S Albert Steffi Riedel-Heller Valur Emilsson Duane Beekly Anne Braae Reinhold Schmidt Deborah Blacker Carlo Masullo Helena Schmidt Rachelle S Doody Gianfranco Spalletta W T Longstreth Thomas J Fairchild Paola Bossù Oscar L Lopez Matthew P Frosch Eleonora Sacchinelli Bernardino Ghetti Qiong Yang Ryan M Huebinger Frank Jessen Shuo Li M Ilyas Kamboh John Morris Oscar Sotolongo-Grau Mindy J Katz Chris Corcoran Melanie Dunstan Amy Braddel Charlene Thomas Alun Meggy Rachel Marshall Amy Gerrish Jade Chapman Miquel Aguilar Sarah Taylor Matt Hill Mònica Díez Fairén Angela Hodges Bruno Vellas Hilkka Soininen Iwona Kloszewska Makrina Daniilidou James Uphill Yogen Patel Joseph T Hughes Jenny Lord James Turton Annette M Hartmann Roberta Cecchetti Chiara Fenoglio Maria Serpente Marina Arcaro Carlo Caltagirone Maria Donata Orfei Antonio Ciaramella Sabrina Pichler Manuel Mayhaus Wei Gu Alberto Lleó Juan Fortea Rafael Blesa Imelda S Barber Keeley Brookes Chiara Cupidi Raffaele Giovanni Maletta David Carrell Sandro Sorbi Susanne Moebus Maria Urbano Alberto Pilotto Johannes Kornhuber Paolo Bosco Stephen Todd David Craig Janet Johnston Michael Gill Brian Lawlor Aoibhinn Lynch Nick C Fox John Hardy Roger L Albin Liana G Apostolova Steven E Arnold Sanjay Asthana Craig S Atwood Clinton T Baldwin Lisa L Barnes Sandra Barral Thomas G Beach James T Becker Eileen H Bigio Thomas D Bird Bradley F Boeve James D Bowen Adam Boxer James R Burke Jeffrey M Burns Joseph D Buxbaum Nigel J Cairns Chuanhai Cao Chris S Carlson Cynthia M Carlsson Regina M Carney Minerva M Carrasquillo Steven L Carroll Carolina Ceballos Diaz Helena C Chui David G Clark David H Cribbs Elizabeth A Crocco Charles DeCarli Malcolm Dick Ranjan Duara Denis A Evans Kelley M Faber Kenneth B Fallon David W Fardo Martin R Farlow Steven Ferris Tatiana M Foroud Douglas R Galasko Marla Gearing Daniel H Geschwind John R Gilbert Neill R Graff-Radford Robert C Green John H Growdon Ronald L Hamilton Lindy E Harrell Lawrence S Honig Matthew J Huentelman Christine M Hulette Bradley T Hyman Gail P Jarvik Erin Abner Lee-Way Jin Gyungah Jun Anna Karydas Jeffrey A Kaye Ronald Kim Neil W Kowall Joel H Kramer Frank M LaFerla James J Lah James B Leverenz Allan I Levey Ge Li Andrew P Lieberman Kathryn L Lunetta Constantine G Lyketsos Daniel C Marson Frank Martiniuk Deborah C Mash Eliezer Masliah Wayne C McCormick Susan M McCurry Andrew N McDavid Ann C McKee Marsel Mesulam Bruce L Miller Carol A Miller Joshua W Miller John C Morris Jill R Murrell Amanda J Myers Sid O'Bryant John M Olichney Vernon S Pankratz Joseph E Parisi Henry L Paulson William Perry Elaine Peskind Aimee Pierce Wayne W Poon Huntington Potter Joseph F Quinn Ashok Raj Murray Raskind Barry Reisberg Christiane Reitz John M Ringman Erik D Roberson Ekaterina Rogaeva Howard J Rosen Roger N Rosenberg Mark A Sager Andrew J Saykin Julie A Schneider Lon S Schneider William W Seeley Amanda G Smith Joshua A Sonnen Salvatore Spina Robert A Stern Russell H Swerdlow Rudolph E Tanzi Tricia A Thornton-Wells John Q Trojanowski Juan C Troncoso Vivianna M Van Deerlin Linda J Van Eldik Harry V Vinters Jean Paul Vonsattel Sandra Weintraub Kathleen A Welsh-Bohmer Kirk C Wilhelmsen Jennifer Williamson Thomas S Wingo Randall L Woltjer Clinton B Wright Chang-En Yu Lei Yu Fabienne Garzia Feroze Golamaully Gislain Septier Sebastien Engelborghs Rik Vandenberghe Peter P De Deyn Carmen Muñoz Fernadez Yoland Aladro Benito Hakan Thonberg Charlotte Forsell Lena Lilius Anne Kinhult-Stählbom Lena Kilander RoseMarie Brundin Letizia Concari Seppo Helisalmi Anne Maria Koivisto Annakaisa Haapasalo Vincent Dermecourt Nathalie Fievet Olivier Hanon Carole Dufouil Alexis Brice Karen Ritchie Bruno Dubois Jayanadra J Himali C Dirk Keene JoAnn Tschanz Annette L Fitzpatrick Walter A Kukull Maria Norton Thor Aspelund Eric B Larson Ron Munger Jerome I Rotter Richard B Lipton María J Bullido Albert Hofman Thomas J Montine Eliecer Coto Eric Boerwinkle Ronald C Petersen Victoria Alvarez Fernando Rivadeneira Eric M Reiman Maura Gallo Christopher J O'Donnell Joan S Reisch Amalia Cecilia Bruni Donald R Royall Martin Dichgans Mary Sano Daniela Galimberti Peter St George-Hyslop Elio Scarpini Debby W Tsuang Michelangelo Mancuso Ubaldo Bonuccelli Ashley R Winslow Antonio Daniele Chuang-Kuo Wu Oliver Peters Benedetta Nacmias Matthias Riemenschneider Reinhard Heun Carol Brayne David C Rubinsztein Jose Bras Rita Guerreiro Ammar Al-Chalabi Christopher E Shaw John Collinge David Mann Magda Tsolaki Jordi Clarimón Rebecca Sussams Simon Lovestone Michael C O'Donovan Michael J Owen Timothy W Behrens Simon Mead Alison M Goate Andre G Uitterlinden Clive Holmes Carlos Cruchaga Martin Ingelsson David A Bennett John Powell Todd E Golde Caroline Graff Philip L De Jager Kevin Morgan Nilufer Ertekin-Taner Onofre Combarros Bruce M Psaty Peter Passmore Steven G Younkin Claudine Berr Vilmundur Gudnason Dan Rujescu Dennis W Dickson Jean-François Dartigues Anita L DeStefano Sara Ortega-Cubero Hakon Hakonarson Dominique Campion Merce Boada John Keoni Kauwe Lindsay A Farrer Christine Van Broeckhoven M Arfan Ikram Lesley Jones Jonathan L Haines Christophe Tzourio Lenore J Launer Valentina Escott-Price Richard Mayeux Jean-François Deleuze Najaf Amin Peter A Holmans Margaret A Pericak-Vance Philippe Amouyel Cornelia M van Duijn Alfredo Ramirez Li-San Wang Jean-Charles Lambert Sudha Seshadri Julie Williams Gerard D Schellenberg

Nat Genet 2017 09 17;49(9):1373-1384. Epub 2017 Jul 17.

Penn Neurodegeneration Genomics Center, Department of Pathology and Laboratory Medicine, University of Pennsylvania Perelman School of Medicine, Philadelphia, Pennsylvania, USA.

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September 2017

Distinct C9orf72-Associated Dipeptide Repeat Structures Correlate with Neuronal Toxicity.

PLoS One 2016 24;11(10):e0165084. Epub 2016 Oct 24.

Department of Neurology, University of Michigan, Ann Arbor, Michigan, United States of America.

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June 2017

Evaluation of Antisense Oligonucleotides Targeting ATXN3 in SCA3 Mouse Models.

Mol Ther Nucleic Acids 2017 Jun 12;7:200-210. Epub 2017 Apr 12.

Department of Neurology, University of Michigan, Ann Arbor, MI 48109-2200, USA. Electronic address:

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June 2017

COMPASS: A computational model to predict changes in MMSE scores 24-months after initial assessment of Alzheimer's disease.

Sci Rep 2016 10 5;6:34567. Epub 2016 Oct 5.

Department of Computational Medicine and Bioinformatics, University of Michigan, USA.

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October 2016

Differential recruitment of UBQLN2 to nuclear inclusions in the polyglutamine diseases HD and SCA3.

Neurobiol Dis 2015 Oct 30;82:281-288. Epub 2015 Jun 30.

Department of Neurology, University of Michigan, Ann Arbor, MI, USA. Electronic address:

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October 2015

RAN translation at CGG repeats induces ubiquitin proteasome system impairment in models of fragile X-associated tremor ataxia syndrome.

Hum Mol Genet 2015 Aug 7;24(15):4317-26. Epub 2015 May 7.

Department of Neurology, University of Michigan, Ann Arbor, MI, USA, Neurology, U.S. Department of Veterans Affairs Medical Center, Ann Arbor, MI, USA

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August 2015

Dominant negative effect of polyglutamine expansion perturbs normal function of ataxin-3 in neuronal cells.

Hum Mol Genet 2015 Jan 20;24(1):100-17. Epub 2014 Aug 20.

Life and Health Sciences Research Institute (ICVS), School of Health Sciences, University of Minho, 4710-057 Braga ICVS/3B's-PT Government Associate Laboratory, Braga/Guimarães and

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January 2015

Intrinsic disorder drives N-terminal ubiquitination by Ube2w.

Nat Chem Biol 2015 Jan 1;11(1):83-9. Epub 2014 Dec 1.

Department of Biochemistry, University of Washington, Seattle, Washington, USA.

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January 2015

Effects of multiple genetic loci on age at onset in late-onset Alzheimer disease: a genome-wide association study.

Authors:
Adam C Naj Gyungah Jun Christiane Reitz Brian W Kunkle William Perry Yo Son Park Gary W Beecham Ruchita A Rajbhandary Kara L Hamilton-Nelson Li-San Wang John S K Kauwe Matthew J Huentelman Amanda J Myers Thomas D Bird Bradley F Boeve Clinton T Baldwin Gail P Jarvik Paul K Crane Ekaterina Rogaeva M Michael Barmada F Yesim Demirci Carlos Cruchaga Patricia L Kramer Nilufer Ertekin-Taner John Hardy Neill R Graff-Radford Robert C Green Eric B Larson Peter H St George-Hyslop Joseph D Buxbaum Denis A Evans Julie A Schneider Kathryn L Lunetta M Ilyas Kamboh Andrew J Saykin Eric M Reiman Philip L De Jager David A Bennett John C Morris Thomas J Montine Alison M Goate Deborah Blacker Debby W Tsuang Hakon Hakonarson Walter A Kukull Tatiana M Foroud Eden R Martin Jonathan L Haines Richard P Mayeux Lindsay A Farrer Gerard D Schellenberg Margaret A Pericak-Vance Marilyn S Albert Roger L Albin Liana G Apostolova Steven E Arnold Robert Barber Lisa L Barnes Thomas G Beach James T Becker Duane Beekly Eileen H Bigio James D Bowen Adam Boxer James R Burke Nigel J Cairns Laura B Cantwell Chuanhai Cao Chris S Carlson Regina M Carney Minerva M Carrasquillo Steven L Carroll Helena C Chui David G Clark Jason Corneveaux David H Cribbs Elizabeth A Crocco Charles DeCarli Steven T DeKosky Malcolm Dick Dennis W Dickson Ranjan Duara Kelley M Faber Kenneth B Fallon Martin R Farlow Steven Ferris Matthew P Frosch Douglas R Galasko Mary Ganguli Marla Gearing Daniel H Geschwind Bernardino Ghetti John R Gilbert Jonathan D Glass John H Growdon Ronald L Hamilton Lindy E Harrell Elizabeth Head Lawrence S Honig Christine M Hulette Bradley T Hyman Gregory A Jicha Lee-Way Jin Anna Karydas Jeffrey A Kaye Ronald Kim Edward H Koo Neil W Kowall Joel H Kramer Frank M LaFerla James J Lah James B Leverenz Allan I Levey Ge Li Andrew P Lieberman Chiao-Feng Lin Oscar L Lopez Constantine G Lyketsos Wendy J Mack Frank Martiniuk Deborah C Mash Eliezer Masliah Wayne C McCormick Susan M McCurry Andrew N McDavid Ann C McKee Marsel Mesulam Bruce L Miller Carol A Miller Joshua W Miller Jill R Murrell John M Olichney Vernon S Pankratz Joseph E Parisi Henry L Paulson Elaine Peskind Ronald C Petersen Aimee Pierce Wayne W Poon Huntington Potter Joseph F Quinn Ashok Raj Murray Raskind Barry Reisberg John M Ringman Erik D Roberson Howard J Rosen Roger N Rosenberg Mary Sano Lon S Schneider William W Seeley Amanda G Smith Joshua A Sonnen Salvatore Spina Robert A Stern Rudolph E Tanzi Tricia A Thornton-Wells John Q Trojanowski Juan C Troncoso Otto Valladares Vivianna M Van Deerlin Linda J Van Eldik Badri N Vardarajan Harry V Vinters Jean Paul Vonsattel Sandra Weintraub Kathleen A Welsh-Bohmer Jennifer Williamson Sarah Wishnek Randall L Woltjer Clinton B Wright Steven G Younkin Chang-En Yu Lei Yu

JAMA Neurol 2014 Nov;71(11):1394-404

Department of Neurological Sciences, Rush University Medical Center, Chicago, Illinois.

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November 2014

Differential effects of delayed aging on phenotype and striatal pathology in a murine model of Huntington disease.

J Neurosci 2014 Nov;34(47):15658-68

Departments of Neurology, Michigan Alzheimer Disease Center, Ann Arbor, Michigan 48105, and Neurology Service and Geriatrics Research, Education, and Clinical Center, VAAAHS, Ann Arbor, Michigan 48105

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November 2014

Alterations in cerebellar physiology are associated with a stiff-legged gait in Atcay(ji-hes) mice.

Neurobiol Dis 2014 Jul 12;67:140-8. Epub 2014 Apr 12.

Department of Neurology, University of Michigan, Ann Arbor, MI 48109, USA. Electronic address:

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July 2014

F-box only protein 2 (Fbxo2) regulates amyloid precursor protein levels and processing.

J Biol Chem 2014 Mar 27;289(10):7038-48. Epub 2014 Jan 27.

From the Department of Neurology, University of Michigan, Ann Arbor, Michigan 48104.

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March 2014

Transcriptional changes and developmental abnormalities in a zebrafish model of myotonic dystrophy type 1.

Dis Model Mech 2014 Jan 2;7(1):143-55. Epub 2013 Oct 2.

Department of Neurology, University of Michigan, Ann Arbor, MI 48109, USA.

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January 2014

New hope for therapy in neurodegenerative diseases.

Cell Res 2013 Oct 23;23(10):1159-60. Epub 2013 Jul 23.

Department of Neurology, University of Michigan, A. Alfred Taubman Medical Research Institute, Ann Arbor, MI 48109-2200, USA.

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October 2013

C9orf72-associated FTD/ALS: when less is more.

Neuron 2013 Oct;80(2):257-8

Department of Neurology, University of Michigan Medical Center, Ann Arbor, MI 48109, USA.

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October 2013

JosD1, a membrane-targeted deubiquitinating enzyme, is activated by ubiquitination and regulates membrane dynamics, cell motility, and endocytosis.

J Biol Chem 2013 Jun 26;288(24):17145-55. Epub 2013 Apr 26.

Department of Neurology, University of Michigan, Ann Arbor, Michigan 48109, USA.

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June 2013

The ubiquitin-conjugating enzyme (E2) Ube2w ubiquitinates the N terminus of substrates.

J Biol Chem 2013 Jun 21;288(26):18784-8. Epub 2013 May 21.

Department of Neurology, University of Michigan, Ann Arbor, Michigan 48109, USA.

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June 2013

Valosin-containing protein (VCP/p97) is an activator of wild-type ataxin-3.

PLoS One 2012 6;7(9):e43563. Epub 2012 Sep 6.

Center for Neuroscience and Cell Biology, University of Coimbra, Coimbra, Portugal.

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May 2013

Analysis of the tau-associated proteome reveals that exchange of Hsp70 for Hsp90 is involved in tau degradation.

ACS Chem Biol 2012 Oct 25;7(10):1677-86. Epub 2012 Jul 25.

Departments of Pathology, University of Michigan, Ann Arbor, Michigan 48103, United States.

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October 2012

Cognitive decline and aging: the role of concussive and subconcussive impacts.

Exerc Sport Sci Rev 2012 Jul;40(3):138-44

School of Kinesiology, University of Michigan at Ann Arbor, Ann Arbor, MI 48109, USA.

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July 2012

Alzheimer's disease and environmental exposure to lead: the epidemiologic evidence and potential role of epigenetics.

Curr Alzheimer Res 2012 Jun;9(5):563-73

University of Michigan, School of Public Health, Department of Environmental Health Sciences, MI, USA.

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June 2012

Establishment of a novel fluorescence-based method to evaluate chaperone-mediated autophagy in a single neuron.

PLoS One 2012 7;7(2):e31232. Epub 2012 Feb 7.

Department of Molecular and Pharmacological Neuroscience, Graduate School of Biomedical Sciences, Hiroshima University, Hiroshima, Japan.

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June 2012

Toward understanding Machado-Joseph disease.

Prog Neurobiol 2012 May 23;97(2):239-57. Epub 2011 Nov 23.

Department of Neurology, University of Michigan, A. Alfred Taubman Biomedical Sciences Research Building-BSRB, 109 Zina Pitcher Place, Ann Arbor, MI 48109-2200, USA.

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May 2012

Compromised mitochondrial complex II in models of Machado-Joseph disease.

Biochim Biophys Acta 2012 Feb 20;1822(2):139-49. Epub 2011 Oct 20.

CNC - Center for Neuroscience and Cell Biology, University of Coimbra, Coimbra, Portugal.

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February 2012

Genetics of dementia.

Semin Neurol 2011 Nov 21;31(5):449-60. Epub 2012 Jan 21.

Department of Neurology, University of Michigan, Ann Arbor, Michigan 48109, USA.

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November 2011

Splice isoform-specific suppression of the Cav2.1 variant underlying spinocerebellar ataxia type 6.

Neurobiol Dis 2011 Sep 29;43(3):533-42. Epub 2011 Apr 29.

Institute of Neuroscience, School of Life Sciences, National Yang-Ming University, Taipei, Taiwan.

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September 2011

Balancing act: deubiquitinating enzymes in the nervous system.

Trends Neurosci 2011 Jul 24;34(7):370-82. Epub 2011 Jun 24.

Wayne State University School of Medicine, Department of Pharmacology and Department of Neurology, 540 E Canfield, Scott Hall Room 6105, Detroit, Michigan 48201, USA.

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July 2011

The Machado-Joseph disease-associated mutant form of ataxin-3 regulates parkin ubiquitination and stability.

Hum Mol Genet 2011 Jan 11;20(1):141-54. Epub 2010 Oct 11.

Centre for Neuronal Survival and McGill Parkinson Program, Department of Neurology and Neurosurgery, Montreal Neurological Institute, McGill University, Montreal, Quebec, Canada.

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January 2011

Histone deacetylases suppress CGG repeat-induced neurodegeneration via transcriptional silencing in models of fragile X tremor ataxia syndrome.

PLoS Genet 2010 Dec 9;6(12):e1001240. Epub 2010 Dec 9.

Department of Neurology, University of Michigan, Ann Arbor, Michigan, United States of America.

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December 2010

Early alterations of autophagy in Huntington disease-like mice.

Autophagy 2010 Nov 16;6(8):1206-8. Epub 2010 Nov 16.

Department of Neurology, University of Michigan, Ann Arbor, MI, USA.

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November 2010

Early autophagic response in a novel knock-in model of Huntington disease.

Hum Mol Genet 2010 Oct 8;19(19):3702-20. Epub 2010 Jul 8.

Department of Neurology, University of Michigan, Ann Arbor, MI 48109-2200, USA.

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October 2010

Splice isoforms of the polyglutamine disease protein ataxin-3 exhibit similar enzymatic yet different aggregation properties.

PLoS One 2010 Oct 27;5(10):e13695. Epub 2010 Oct 27.

Graduate Program in Molecular and Cellular Biology, University of Iowa, Iowa City, Iowa, United States of America.

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October 2010

Understanding the role of the Josephin domain in the PolyUb binding and cleavage properties of ataxin-3.

PLoS One 2010 Aug 26;5(8):e12430. Epub 2010 Aug 26.

National Institute for Medical Research, Medical Research Council, London, United Kingdom.

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August 2010

Ataxin-3 plays a role in mouse myogenic differentiation through regulation of integrin subunit levels.

PLoS One 2010 Jul 23;5(7):e11728. Epub 2010 Jul 23.

Life and Health Sciences Research Institute (ICVS), School of Health Sciences, University of Minho, Braga, Portugal.

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July 2010

Conditional Niemann-Pick C mice demonstrate cell autonomous Purkinje cell neurodegeneration.

Hum Mol Genet 2010 Mar 10;19(5):837-47. Epub 2009 Dec 10.

Department of Pathology, University of Michigan, Ann Arbor, MI 48109-0605, USA.

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March 2010

RNA-mediated neurodegeneration in repeat expansion disorders.

Ann Neurol 2010 Mar;67(3):291-300

Department of Neurology, University of Michigan, Ann Arbor, MI 48109, USA.

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March 2010

Caring for Machado-Joseph disease: current understanding and how to help patients.

Parkinsonism Relat Disord 2010 Jan 6;16(1):2-7. Epub 2009 Oct 6.

Department of Neurology, University of Campinas (UNICAMP), Campinas, São Paulo, Brazil.

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January 2010

Physiologic alterations in ataxia: channeling changes into novel therapies.

Arch Neurol 2009 Oct;66(10):1196-201

Department of Neurology, University of Michigan, Ann Arbor,Michigan, USA.

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October 2009

Allele-specific RNAi mitigates phenotypic progression in a transgenic model of Alzheimer's disease.

Mol Ther 2009 Sep 16;17(9):1563-73. Epub 2009 Jun 16.

Department of Neurology, University of Michigan, Ann Arbor, Michigan, USA.

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September 2009

The spinocerebellar ataxias.

Authors:
Henry L Paulson

J Neuroophthalmol 2009 Sep;29(3):227-37

Department of Neurology, University of Michigan Health Systems, Ann Arbor, MI 48109, USA.

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September 2009

In vivo suppression of polyglutamine neurotoxicity by C-terminus of Hsp70-interacting protein (CHIP) supports an aggregation model of pathogenesis.

Neurobiol Dis 2009 Mar 8;33(3):342-53. Epub 2008 Nov 8.

Graduate Program in Neuroscience and Medical Scientist Training Program, University of Iowa, 2206 MERF, Iowa City, IA 52242, USA.

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March 2009

Ubiquitination directly enhances activity of the deubiquitinating enzyme ataxin-3.

EMBO J 2009 Feb 15;28(4):372-82. Epub 2009 Jan 15.

Department of Neurology, University of Michigan, Ann Arbor, MI 48109, USA.

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February 2009

Obsessive and compulsive symptoms in prediagnosed Huntington's disease.

J Clin Psychiatry 2008 Nov 9;69(11):1758-65. Epub 2008 Sep 9.

University of Iowa, Department of Psychiatry, MEB 1-321, Iowa City, IA 52242-1000, USA.

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November 2008

Polyglutamine neurodegeneration: protein misfolding revisited.

Trends Neurosci 2008 Oct 6;31(10):521-8. Epub 2008 Sep 6.

Program in Neuroscience and Medical Scientist Training Program, University of Iowa, 2206 MERF, Iowa City, IA 52242, USA.

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October 2008

Diversity in tissue expression, substrate binding, and SCF complex formation for a lectin family of ubiquitin ligases.

J Biol Chem 2008 May 18;283(19):12717-29. Epub 2008 Jan 18.

Veterans Affairs Medical Center, Iowa City, Iowa 52242, USA.

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May 2008

Risperidone and the treatment of psychiatric, motor, and cognitive symptoms in Huntington's disease.

Ann Clin Psychiatry 2008 Jan-Mar;20(1):1-3

Department of Psychiatry, University of Iowa, Iowa City, IA 52242-1000, USA.

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April 2008

Cellular turnover of the polyglutamine disease protein ataxin-3 is regulated by its catalytic activity.

J Biol Chem 2007 Oct 10;282(40):29348-58. Epub 2007 Aug 10.

Department of Neurology, University of Iowa, Iowa City, Iowa 52242, USA.

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October 2007

Spinocerebellar ataxias: an update.

Curr Opin Neurol 2007 Aug;20(4):438-46

Department of Neurology, National Yang-Ming University School of Medicine, The Neurological Institute, Taipei Veterans General Hospital, Taipei, Taiwan.

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August 2007

Technology insight: therapeutic RNA interference--how far from the neurology clinic?

Nat Clin Pract Neurol 2007 Jul;3(7):394-404

University of Iowa, Iowa City, IA, USA.

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July 2007

Selective cochlear degeneration in mice lacking the F-box protein, Fbx2, a glycoprotein-specific ubiquitin ligase subunit.

J Neurosci 2007 May;27(19):5163-71

Medical Scientist Training Program, University of Iowa Roy J. and Lucille A. Carver College of Medicine, Iowa City, Iowa 52242, USA.

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May 2007

Dominantly inherited ataxias: lessons learned from Machado-Joseph disease/spinocerebellar ataxia type 3.

Authors:
Henry L Paulson

Semin Neurol 2007 Apr;27(2):133-42

Department of Neurology, University of Iowa College of Medicine, Iowa City, Iowa 52242, USA.

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April 2007

Dangerous liaisons: polyglutamine meets HMGB.

Nat Cell Biol 2007 Apr;9(4):359-61

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April 2007

A novel route for F-box protein-mediated ubiquitination links CHIP to glycoprotein quality control.

J Biol Chem 2006 Jul 8;281(29):20242-51. Epub 2006 May 8.

Medical Scientist Training Program, Graduate Program in Neuroscience, University of Iowa Roy J. and Lucille A. Carver College of Medicine, Iowa City, Iowa 52242, USA.

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July 2006

If it's not one thing, it's another.

Authors:
Henry L Paulson

Nat Genet 2006 Jul;38(7):743-4

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July 2006

Brain structure in preclinical Huntington's disease.

Biol Psychiatry 2006 Jan 22;59(1):57-63. Epub 2005 Aug 22.

Department of Psychiatry, University of Iowa Roy and Lucille Carver College of Medicine, Iowa City, Iowa 52242, USA.

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January 2006

RNA interference in neuroscience: progress and challenges.

Cell Mol Neurobiol 2005 Dec;25(8):1195-207

Department of Neurology, University of Iowa Roy J. and Lucille A. Carver College of Medicine, Iowa City, IA 52242-1101, USA.

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December 2005

Silencing primary dystonia: lentiviral-mediated RNA interference therapy for DYT1 dystonia.

J Neurosci 2005 Nov;25(45):10502-9

Department of Neurology, Carver College of Medicine, University of Iowa, Iowa City, Iowa 52242, USA.

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November 2005

CHIP suppresses polyglutamine aggregation and toxicity in vitro and in vivo.

J Neurosci 2005 Oct;25(40):9152-61

Department of Neurology, University of Iowa, Roy J. and Lucille A. Carver College of Medicine, Iowa City, Iowa 52242, USA.

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October 2005

Defining the role of ubiquitin-interacting motifs in the polyglutamine disease protein, ataxin-3.

J Biol Chem 2005 Sep 21;280(36):32026-34. Epub 2005 Jul 21.

Neuroscience Graduate Program, University of Iowa, Iowa City 52242, USA.

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September 2005

Breaks in coordination: DNA repair in inherited ataxia.

Neuron 2005 Jun;46(6):845-8

Department of Neurology and Carver College of Medicine, University of Iowa, Iowa City, Iowa 52245, USA.

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June 2005

Ataxin-3 suppresses polyglutamine neurodegeneration in Drosophila by a ubiquitin-associated mechanism.

Mol Cell 2005 Apr;18(1):37-48

Department of Biology, University of Pennsylvania, 415 South University Avenue, Philadelphia, Pennsylvania 19104, USA.

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April 2005

RNA interference improves motor and neuropathological abnormalities in a Huntington's disease mouse model.

Proc Natl Acad Sci U S A 2005 Apr 5;102(16):5820-5. Epub 2005 Apr 5.

Program in Gene Therapy, Department of Internal Medicine, University of Iowa, Iowa City, IA 52242, USA.

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April 2005

Caspase-mediated proteolysis of the polyglutamine disease protein ataxin-3.

J Neurochem 2004 May;89(4):908-18

Neuroscience Graduate Program and Department of Neurology, University of Iowa, Iowa City, Iowa 52242, USA.

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May 2004

Targeting Alzheimer's disease genes with RNA interference: an efficient strategy for silencing mutant alleles.

Nucleic Acids Res 2004 30;32(2):661-8. Epub 2004 Jan 30.

Department of Neurology, Graduate Program in Genetics, University of Iowa Roy J. and Lucille A. Carver College of Medicine, Iowa City, IA 52242, USA.

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April 2004

Molecular medicine for the brain: silencing of disease genes with RNA interference.

Lancet Neurol 2004 Mar;3(3):145-9

Department of Internal Medicine, University of Iowa, Iowa City, IA 52242, USA.

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March 2004

Aberrant cellular behavior of mutant torsinA implicates nuclear envelope dysfunction in DYT1 dystonia.

J Neurosci 2004 Mar;24(11):2593-601

Department of Neurology, Carver College of Medicine at the University of Iowa, Iowa City, Iowa 52242, USA.

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March 2004

Poly-ubiquitin binding by the polyglutamine disease protein ataxin-3 links its normal function to protein surveillance pathways.

J Biol Chem 2004 Jan 5;279(5):3605-11. Epub 2003 Nov 5.

Department of Neurology, Carver College of Medicine, University of Iowa, Iowa City, Iowa 52242-1101, USA.

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January 2004

Destabilization of a non-pathological variant of ataxin-3 results in fibrillogenesis via a partially folded intermediate: a model for misfolding in polyglutamine disease.

J Mol Biol 2004 Jan;335(1):333-41

Department of Biochemistry and Molecular Biology, Structural Biology Group, Monash University, Clayton Campus, PO Box 13D, Wellington Rd., 3800, Clayton, Vic., Australia.

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Protein aggregation and the ubiquitin proteasome pathway: gaining the UPPer hand on neurodegeneration.

Curr Opin Genet Dev 2003 Jun;13(3):253-61

Department of Neurology and Neuroscience Graduate Program, Paulson Laboratory - 240 EMRB, University of Iowa, Iowa City, Iowa 52242, USA.

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June 2003