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Steven Hersch

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Vanita Chopra; Patricia M Cipicchio; Christina Edgerly; Robert J Ferrante; Steven M Hersch; Jinho Kim; Molly A Lauver; K Lakshmi Narayanan; Luisa Quinti; Richard B Silverman; Lorraine Vollor; Soo Hyuk Choi; Aleksey G Kazantsev
The sirtuin 2 inhibitor AK-7 is neuroprotective in Huntington's disease mouse models.
H Diana Rosas; David H Salat; Ken K Kwong; Ashley Bush; Nan-Kuei Chen; Y Iris Chen; Gheorghe Doros; Jonathan Fox; Steven M Hersch
Alterations in brain transition metals in Huntington disease: an evolving and intricate story.
Jong-Min Lee; Diane Lucente; Marcy E MacDonald; Karen Marder; Russell L Margolis; Elizabeth McCusker; Patrick J Morrison; Richard H Myers; Jayalakshmi Srinidhi Mysore; Martha Nance; Andrea Novelletto; Eliana Marisa Ramos; Herminia D Rosas; Christopher A Ross; Marie-Helene Saint-Hilaire; Jorge Sequeiros; Ferdinando Squitieri; Oksana Suchowersky; Ronald J Trent; Andrea Zanko; Ruth K Abramson; Tetsuo Ashizawa; Carmen Ayuso; Stefano Di Donato; Samuel Frank; Marina Frontali; Tammy Gillis; Estrella Gomez-Tortosa; Annamaria Griguoli; Madaline B Harrison; Michael R Hayden; Steven M Hersch; Randi Jones; James F Gusella
Common SNP-based haplotype analysis of the 4p16.3 Huntington disease gene region.

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All Publications

2012: Vanita Chopra; Patricia M Cipicchio; Christina Edgerly; Robert J Ferrante; Steven M Hersch; Jinho Kim; Molly A Lauver; K Lakshmi Narayanan; Luisa Quinti; Richard B Silverman; Lorraine Vollor; Soo Hyuk Choi; Aleksey G Kazantsev
The sirtuin 2 inhibitor AK-7 is neuroprotective in Huntington's disease mouse models.
Cell reports 2012;2(6):1492-7.
2012: H Diana Rosas; David H Salat; Ken K Kwong; Ashley Bush; Nan-Kuei Chen; Y Iris Chen; Gheorghe Doros; Jonathan Fox; Steven M Hersch
Alterations in brain transition metals in Huntington disease: an evolving and intricate story.
Archives of neurology 2012;69(7):887-93.
2012: Jong-Min Lee; Diane Lucente; Marcy E MacDonald; Karen Marder; Russell L Margolis; Elizabeth McCusker; Patrick J Morrison; Richard H Myers; Jayalakshmi Srinidhi Mysore; Martha Nance; Andrea Novelletto; Eliana Marisa Ramos; Herminia D Rosas; Christopher A Ross; Marie-Helene Saint-Hilaire; Jorge Sequeiros; Ferdinando Squitieri; Oksana Suchowersky; Ronald J Trent; Andrea Zanko; Ruth K Abramson; Tetsuo Ashizawa; Carmen Ayuso; Stefano Di Donato; Samuel Frank; Marina Frontali; Tammy Gillis; Estrella Gomez-Tortosa; Annamaria Griguoli; Madaline B Harrison; Michael R Hayden; Steven M Hersch; Randi Jones; James F Gusella
Common SNP-based haplotype analysis of the 4p16.3 Huntington disease gene region.
American journal of human genetics 2012;90(3):434-44.
2012: J-M Lee; D Lucente; Marcy E MacDonald; K Marder; Russell L Margolis; E McCusker; P Morrison; R H Myers; J S Mysore; M Nance; A Novelletto; S Orobello; Jane S Paulsen; E M Ramos; H D Rosas; Christopher A Ross; M H Saint-Hilaire; J Sequeiros; F Squitieri; O Suchowersky; R J A Trent; S C Warby; A Zanko; R K Abramson; T Ashizawa; C Ayuso; S Di Donato; S Frank; M Frontali; T Gillis; E Gomez-Tortosa; J F Gusella; M B Harrison; Michael R Hayden; Steven M Hersch; R Jones; G B Landwehrmeyer; J-H Lee
CAG repeat expansion in Huntington disease determines age at onset in a fully dominant fashion.
Neurology 2012;78(10):690-5.
2011: Michele M Maxwell; Johnathan Nobles; Luisa Quinti; Elizabeth M Tomkinson; John W Wizeman; Allison M Amore; Vanita Chopra; Steven M Hersch; Aleksey G Kazantsev
The Sirtuin 2 microtubule deacetylase is an abundant neuronal protein that accumulates in the aging CNS.
Human molecular genetics 2011;20(20):3986-96.
2011: Yi Hu; Zhixiang Liao; Joseph J Locascio; Wayne R Matson; H Diana Rosas; Raman Chopra; Vanita Chopra; Hongliu Ding; Robert J Ferrante; Steven M Hersch; Bin Zheng; Clemens R Scherzer
Transcriptional modulator H2A histone family, member Y (H2AFY) marks Huntington disease activity in man and mouse.
Proceedings of the National Academy of Sciences of the United States of America 2011;108(41):17141-6.
2011: H Diana Rosas; David H Salat; Tyler Triggs; Gheorghe Doros; Bruce Fischl; Stephanie Y Lee; Keith Malarick; Martin Reuter; Steven M Hersch
A tale of two factors: what determines the rate of progression in Huntington's disease? A longitudinal MRI study.
Movement disorders : official journal of the Movement Disorder Society 2011;26(9):1691-7.
2011: Jonathan H Fox; Teal Connor; Megan Stiles; Jibrin Kama; Zhen Lu; Kathryn Dorsey; Gregory Liebermann; Ellen Sapp; Robert A Cherny; Mary Banks; Irene Volitakis; Marian DiFiglia; Oksana Berezovska; Ashley I Bush; Gregory Lieberman; Steven M Hersch
Cysteine oxidation within N-terminal mutant huntingtin promotes oligomerization and delays clearance of soluble protein.
The Journal of biological chemistry 2011;286(20):18320-30.
2011: Andreas Weiss; Stephan Grueninger; Dorothee Abramowski; Francesco Paolo Di Giorgio; Miriam Moscovitch Lopatin; H Diana Rosas; Steven M Hersch; Paolo Paganetti
Microtiter plate quantification of mutant and wild-type huntingtin normalized to cell count.
Analytical biochemistry 2011;410(2):304-6.
2010: Jinho Kim; Daniel J Amante; Jennifer P Moody; Christina K Edgerly; Olivia L Bordiuk; Karen Smith; Samantha Matson; Wayne R Matson; Clemens R Scherzer; H Diana Rosas; Steven M Hersch; Robert J Ferrante
Reduced creatine kinase as a central and peripheral biomarker in Huntington's disease.
Biochimica et biophysica acta 2010;1802(7-8):673-81.
2010: Erika N Ebbel; Nancy Leymarie; Susan Schiavo; Swati Sharma; Sona Gevorkian; Steven M Hersch; Wayne R Matson; Catherine E Costello
Identification of phenylbutyrate-generated metabolites in Huntington disease patients using parallel liquid chromatography/electrochemical array/mass spectrometry and off-line tandem mass spectrometry.
Analytical biochemistry 2010;399(2):152-61.
2010: H Diana Rosas; Stephanie Y Lee; Alexander C Bender; Alexandra K Zaleta; Mark Vangel; Peng Yu; Bruce Fischl; Vasanth Pappu; Christina Onorato; Jang-Ho Cha; David H Salat; Steven M Hersch
Altered white matter microstructure in the corpus callosum in Huntington's disease: implications for cortical "disconnection".
NeuroImage 2010;49(4):2995-3004.
2010: Miriam Moscovitch-Lopatin; Andreas Weiss; H Diana Rosas; James Ritch; Gheorghe Doros; Kimberly B Kegel; Marian DiFiglia; Rainer Kuhn; Graeme Bilbe; Paolo Paganetti; Steven M Hersch
Optimization of an HTRF Assay for the Detection of Soluble Mutant Huntingtin in Human Buffy Coats: A Potential Biomarker in Blood for Huntington Disease.
PLoS currents 2010;2():RRN1205.
2010: Luisa Quinti; Vanita Chopra; dante rotili; Sergio Valente; Allison Amore; Gianluigi Franci; Sarah Meade; Marta Valenza; Lucia Altucci; Michele M Maxwell; Elena Cattaneo; Steven M Hersch; Antonello Mai; Aleksey Kazantsev
Evaluation of histone deacetylases as drug targets in Huntington's disease models. Study of HDACs in brain tissues from R6/2 and CAG140 knock-in HD mouse models and human patients and in a neuronal HD cell model.
PLoS currents 2010;2():.
2010: Jonathan H Fox; Teal Connor; Vanita Chopra; Kate Dorsey; Jibrin A Kama; Dorothee Bleckmann; Claudia Betschart; Daniel Hoyer; Stefan Frentzel; Marian DiFiglia; Paolo Paganetti; Steven M Hersch
The mTOR kinase inhibitor Everolimus decreases S6 kinase phosphorylation but fails to reduce mutant huntingtin levels in brain and is not neuroprotective in the R6/2 mouse model of Huntington's disease.
Molecular neurodegeneration 2010;5():26.
2009: Andreas Weiss; Dorothee Abramowski; Miriam Bibel; Ruth Bodner; Vanita Chopra; Marian DiFiglia; Jonathan Fox; Kimberly Kegel; Corinna Klein; Stephan Grueninger; Steven M Hersch; David Housman; Etienne Régulier; H Diana Rosas; Muriel Stefani; Scott Zeitlin; Graeme Bilbe; Paolo Paganetti
Single-step detection of mutant huntingtin in animal and human tissues: a bioassay for Huntington's disease.
Analytical biochemistry 2009;395(1):8-15.
2009: D Brocklebank; Javier Gayán; J M Andresen; S A Roberts; Anne B Young; S R Snodgrass; J B Penney; M A Ramos-Arroyo; J J Cha; H D Rosas; Steven M Hersch; A Feigin; Stacey Cherny; N S Wexler; David E Housman; Lon R Cardon
Repeat instability in the 27-39 CAG range of the HD gene in the Venezuelan kindreds: Counseling implications.
American journal of medical genetics. Part B, Neuropsychiatric genetics : the official publication of the International Society of Psychiatric Genetics 2009;150B(3):425-9.
2008: H Diana Rosas; David H Salat; Stephanie Y Lee; Alexandra K Zaleta; Nathanael Hevelone; Steven M Hersch
Complexity and heterogeneity: what drives the ever-changing brain in Huntington's disease?
Annals of the New York Academy of Sciences 2008;1147():196-205.
2008: Wanzhao Liu; Lori A Kennington; H Diana Rosas; Steven M Hersch; Jang-Ho Cha; Phillip D Zamore; Neil Aronin
Linking SNPs to CAG repeat length in Huntington's disease patients.
Nature methods 2008;5(11):951-3.
2008: Susan Schiavo; Erika Ebbel; Swati Sharma; Wayne Matson; Bruce Kristal; Steven M Hersch; Paul Vouros
Metabolite identification using a nanoelectrospray LC-EC-array-MS integrated system.
Analytical chemistry 2008;80(15):5912-23.
2008: Steven M Hersch; H Diana Rosas
Neuroprotection for Huntington's disease: ready, set, slow.
Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics 2008;5(2):226-36.
2008: H Diana Rosas; David H Salat; Stephanie Y Lee; Alexandra K Zaleta; Vasanth Pappu; Bruce Fischl; Doug Greve; Nathanael Hevelone; Steven M Hersch
Cerebral cortex and the clinical expression of Huntington's disease: complexity and heterogeneity.
Brain : a journal of neurology 2008;131(Pt 4):1057-68.
2007: Edward C Stack; Alpaslan Dedeoglu; Karen M Smith; Kerry Cormier; James K Kubilus; Mikhail B Bogdanov; Wayne R Matson; Lichuan Yang; Bruce G Jenkins; Ruth Luthi-Carter; Neil W Kowall; Steven M Hersch; M Flint Beal; Robert J Ferrante
Neuroprotective effects of synaptic modulation in Huntington's disease R6/2 mice.
The Journal of neuroscience : the official journal of the Society for Neuroscience 2007;27(47):12908-15.
2007: Aleksey G Kazantsev; Steven M Hersch
Drug targeting of dysregulated transcription in Huntington's disease.
Progress in neurobiology 2007;83(4):249-59.
2007: Vanita Chopra; Jonathan H Fox; Greg Lieberman; Kathryn Dorsey; Wayne R Matson; Peter Waldmeier; David E Housman; Aleksey G Kazantsev; Anne B Young; Steven M Hersch
A small-molecule therapeutic lead for Huntington's disease: preclinical pharmacology and efficacy of C2-8 in the R6/2 transgenic mouse.
Proceedings of the National Academy of Sciences of the United States of America 2007;104(42):16685-9.
2007: Ghazaleh Sadri-Vakili; Bérengère Bouzou; Caroline L Benn; Mee-Ohk Kim; Prianka Chawla; Ryan P Overland; Kelly E Glajch; Eva Xia; Zhihua Qiu; Steven M Hersch; Timothy Clark; George J Yohrling; Jang-Ho Cha
Histones associated with downregulated genes are hypo-acetylated in Huntington's disease models.
Human molecular genetics 2007;16(11):1293-306.
2007: Cheryl Erwin; Steven M Hersch
Monitoring reportable events and unanticipated problems: the PHAROS and PREDICT studies of Huntington disease.
IRB 2007;29(3):11-6.
2007: Jonathan H Fox; Jibrin A Kama; Gregory Lieberman; Raman Chopra; Kate Dorsey; Vanita Chopra; Irene Volitakis; Robert A Cherny; Ashley I Bush; Steven M Hersch
Mechanisms of copper ion mediated Huntington's disease progression.
PloS one 2007;2(3):e334.
2006: H Diana Rosas; David S Tuch; Nathanael Hevelone; Alexandra K Zaleta; Mark Vangel; Steven M Hersch; David H Salat
Diffusion tensor imaging in presymptomatic and early Huntington's disease: Selective white matter pathology and its relationship to clinical measures.
Movement disorders : official journal of the Movement Disorder Society 2006;21(9):1317-25.
2006: Stephen M Altmann; Andrey Muryshev; Elisa Fossale; Michele M Maxwell; Fran Norflus; Jonathan Fox; Steven M Hersch; Anne B Young; Marcy E MacDonald; Ruben Abagyan; Aleksey G Kazantsev
Discovery of bioactive small-molecule inhibitor of poly adp-ribose polymerase: implications for energy-deficient cells.
Chemistry & biology 2006;13(7):765-70.
2006: Zhihua Qiu; Fran Norflus; Bhupinder Singh; Mary K Swindell; Rodica Buzescu; Michelle Bejarano; Raman Chopra; Birgit Zucker; Caroline L Benn; Derek P DiRocco; Jang-Ho Cha; Robert J Ferrante; Steven M Hersch
Sp1 is up-regulated in cellular and transgenic models of Huntington disease, and its reduction is neuroprotective.
The Journal of biological chemistry 2006;281(24):16672-80.
2006: Steven M Hersch; S Gevorkian; Karen Marder; C Moskowitz; A Feigin; M Cox; P Como; C Zimmerman; M Lin; L Zhang; Aziz Ulug; M Flint Beal; Wayne R Matson; Mikhail B Bogdanov; E Ebbel; Alexandra K Zaleta; Y Kaneko; B Jenkins; Nathanael Hevelone; Hui Zhang; H Yu; D Schoenfeld; Robert J Ferrante; H Diana Rosas
Creatine in Huntington disease is safe, tolerable, bioavailable in brain and reduces serum 8OH2'dG.
Neurology 2006;66(2):250-2.
2006: Jian-Liang Li; Michael R Hayden; Simon Warby; Alexandra Durr; Patrick J Morrison; Martha Nance; Christopher A Ross; Russell L Margolis; Adam Rosenblatt; Ferdinando Squitieri; Luigi Frati; Estrella Gómez-Tortosa; Carmen Ayuso García; Oksana Suchowersky; Mary Lou Klimek; Ronald J A Trent; Elizabeth McCusker; Andrea Novelletto; Marina Frontali; Jane S Paulsen; Randi Jones; Tetsuo Ashizawa; Alice Lazzarini; Vanessa C Wheeler; Ranjana Prakash; Gang Xu; Luc Djoussé; Jayalakshmi Srinidhi Mysore; Tammy Gillis; Michael Hakky; L Adrienne Cupples; Marie H Saint-Hilaire; Jang-Ho Cha; Steven M Hersch; John B Penney; Madaline B Harrison; Susan L Perlman; Andrea Zanko; Ruth K Abramson; Anthony J Lechich; Ayana Duckett; Karen Marder; P Michael Conneally; James Gusella; Marcy E MacDonald; Richard H Myers
Genome-wide significance for a modifier of age at neurological onset in Huntington's disease at 6q23-24: the HD MAPS study.
BMC medical genetics 2006;7():71.
2005: Hoon Ryu; H Diana Rosas; Steven M Hersch; Robert J Ferrante
The therapeutic role of creatine in Huntington's disease.
Pharmacology & therapeutics 2005;108(2):193-207.
2005: Edward C Stack; James K Kubilus; Karen Smith; Kerry Cormier; Steven J Del Signore; Emmanuel Guelin; Hoon Ryu; Steven M Hersch; Robert J Ferrante
Chronology of behavioral symptoms and neuropathological sequela in R6/2 Huntington's disease transgenic mice.
The Journal of comparative neurology 2005;490(4):354-70.
2005: Fran Borovecki; Luca Lovrecic; J Zhou; Hyunkyung Jeong; F Then; H Diana Rosas; Steven M Hersch; Penelope Hogarth; Bérengère Bouzou; Roderick V Jensen; Dimitri Krainc
Genome-wide expression profiling of human blood reveals biomarkers for Huntington's disease.
Proceedings of the National Academy of Sciences of the United States of America 2005;102(31):11023-8.
2005: Basant K Puri; Blair R Leavitt; Michael R Hayden; Christopher A Ross; Adam Rosenblatt; J Timothy Greenamyre; Steven M Hersch; Krishna S Vaddadi; A Sword; David F Horrobin; Mehar Manku; Harald Murck
Ethyl-EPA in Huntington disease: a double-blind, randomized, placebo-controlled trial.
Neurology 2005;65(2):286-92.
2004: Michael A Christie; Steven M Hersch
Demonstration of nondeclarative sequence learning in mice: development of an animal analog of the human serial reaction time task.
Learning & memory (Cold Spring Harbor, N.Y.) 2004;11(6):720-3.
2004: Fran Norflus; Agnes Nanje; Claire-Anne Gutekunst; Geming Shi; Joshua Cohen; Michelle Bejarano; Jonathan Fox; Robert J Ferrante; Steven M Hersch
Anti-inflammatory treatment with acetylsalicylate or rofecoxib is not neuroprotective in Huntington's disease transgenic mice.
Neurobiology of disease 2004;17(2):319-25.
2004: Jonathan H Fox; David S Barber; Bhupinder Singh; Birgit Zucker; Mary K Swindell; Fran Norflus; Rodica Buzescu; Raman Chopra; Robert J Ferrante; Aleksey G Kazantsev; Steven M Hersch
Cystamine increases L-cysteine levels in Huntington's disease transgenic mouse brain and in a PC12 model of polyglutamine aggregation.
Journal of neurochemistry 2004;91(2):413-22.
2004: Steven M Hersch; Robert J Ferrante
Translating therapies for Huntington's disease from genetic animal models to clinical trials.
NeuroRx : the journal of the American Society for Experimental NeuroTherapeutics 2004;1(3):298-306.
2004: H Diana Rosas; A S Feigin; Steven M Hersch
Using advances in neuroimaging to detect, understand, and monitor disease progression in Huntington's disease.
NeuroRx : the journal of the American Society for Experimental NeuroTherapeutics 2004;1(2):263-72.
2004: Nancy S Wexler; Judith Lorimer; Julie Porter; Fidela Gomez; Carol Moskowitz; Edith Shackell; Karen Marder; Graciela Penchaszadeh; Simone A Roberts; Javier Gayán; Denise Brocklebank; Stacey Cherny; Lon R Cardon; Jacqueline Gray; Stephen R Dlouhy; Sandra Wiktorski; Marion E Hodes; P Michael Conneally; Jack B Penney; James Gusella; Jang-Ho Cha; Michael C Irizarry; Diana Rosas; Steven M Hersch; Zane Hollingsworth; Marcy E MacDonald; Anne B Young; J Michael Andresen; David E Housman; Margot Mieja De Young; Ernesto Bonilla; Theresa Stillings; Americo Negrette; S Robert Snodgrass; Maria Dolores Martinez-Jaurrieta; Maria A Ramos-Arroyo; Jacqueline Bickham; Juan Sanchez Ramos; Frederick John Marshall; Ira Shoulson; Gustavo J Rey; Andrew Feigin; Norman Arnheim; Amarilis Acevedo-Cruz; Leticia Acosta; Jose Alvir; Kenneth H Fischbeck; Leslie M Thompson; Angela Young; Leon Dure; Christopher J O'Brien; Jane S Paulsen; Adam Brickman; Denise Krch; Shelley Peery; Penelope Hogarth; Donald S Higgins; Bernhard Landwehrmeyer
Venezuelan kindreds reveal that genetic and environmental factors modulate Huntington's disease age of onset.
Proceedings of the National Academy of Sciences of the United States of America 2004;101(10):3498-503.
2003: Steven M Hersch; Klaus Fink; Jean-Paul Vonsattel; Robert M Friedlander
Minocycline is protective in a mouse model of Huntington's disease.
Annals of neurology 2003;54(6):841; author reply 842-3.
2003: Robert J Ferrante; James K Kubilus; Junghee Lee; Hoon Ryu; Ayshe Beesen; Birgit Zucker; Karen Smith; Neil W Kowall; Rajiv R Ratan; Ruth Luthi-Carter; Steven M Hersch
Histone deacetylase inhibition by sodium butyrate chemotherapy ameliorates the neurodegenerative phenotype in Huntington's disease mice.
The Journal of neuroscience : the official journal of the Society for Neuroscience 2003;23(28):9418-27.
2003: Jian-Liang Li; Michael R Hayden; Elisabeth W Almqvist; Ryan R Brinkman; Alexandra Durr; Catherine Dodé; Patrick J Morrison; Oksana Suchowersky; Christopher A Ross; Russell L Margolis; Adam Rosenblatt; Estrella Gómez-Tortosa; David Mayo Cabrero; Andrea Novelletto; Marina Frontali; Martha Nance; Ronald J A Trent; Elizabeth McCusker; Randi Jones; Jane S Paulsen; Madeline Harrison; Andrea Zanko; Ruth K Abramson; Ana L Russ; Beth Knowlton; Luc Djoussé; Jayalakshmi S Mysore; Suzanne Tariot; Michael F Gusella; Vanessa C Wheeler; Larry D Atwood; L Adrienne Cupples; Marie Saint-Hilaire; Jang-Ho J Cha; Steven M Hersch; Walter J Koroshetz; James Gusella; Marcy E MacDonald; Richard H Myers
A genome scan for modifiers of age at onset in Huntington disease: The HD MAPS study.
American journal of human genetics 2003;73(3):682-7.
2003: Steven M Hersch
Huntington's disease: prospects for neuroprotective therapy 10 years after the discovery of the causative genetic mutation.
Current opinion in neurology 2003;16(4):501-6.
2003: Alpaslan Dedeoglu; James K Kubilus; Lichuan Yang; Kimberly L Ferrante; Steven M Hersch; M Flint Beal; Robert J Ferrante
Creatine therapy provides neuroprotection after onset of clinical symptoms in Huntington's disease transgenic mice.
Journal of neurochemistry 2003;85(6):1359-67.
2003: Barbara L Apostol; Aleksey G Kazantsev; Simona Raffioni; Katalin Illes; Judit Pallos; Laszlo Bodai; Natalia Slepko; James E Bear; Frank B Gertler; Steven M Hersch; David E Housman; J Lawrence Marsh; Leslie Michels Thompson
A cell-based assay for aggregation inhibitors as therapeutics of polyglutamine-repeat disease and validation in Drosophila.
Proceedings of the National Academy of Sciences of the United States of America 2003;100(10):5950-5.
2002: Roshni R Singaraja; Shinji Hadano; Martina Metzler; Scott Givan; Cheryl L Wellington; Simon Warby; Anat Yanai; Claire-Anne Gutekunst; Blair R Leavitt; Hong Yi; Keith Fichter; Lu Gan; Krista McCutcheon; Vikramjit Chopra; Jennifer Michel; Steven M Hersch; Joh-E Ikeda; Michael R Hayden
HIP14, a novel ankyrin domain-containing protein, links huntingtin to intracellular trafficking and endocytosis.
Human molecular genetics 2002;11(23):2815-28.
2002: William T Dauer; Nikolai Kholodilov; Miquel Vila; Anne-Cecile Trillat; Rose E Goodchild; Kristin Larsen; Roland G W Staal; Kim Tieu; Yvonne Schmitz; Chao Annie Yuan; Marcelo Rocha; Vernice Jackson-Lewis; Steven M Hersch; David Sulzer; Serge Przedborski; Robert Burke; René Hen
Resistance of alpha -synuclein null mice to the parkinsonian neurotoxin MPTP.
Proceedings of the National Academy of Sciences of the United States of America 2002;99(22):14524-9.
2002: Alpaslan Dedeoglu; James K Kubilus; Thomas M Jeitner; Samantha Matson; Mikhail B Bogdanov; Neil W Kowall; Wayne R Matson; Arthur J L Cooper; Rajiv R Ratan; M Flint Beal; Steven M Hersch; Robert J Ferrante
Therapeutic effects of cystamine in a murine model of Huntington's disease.
The Journal of neuroscience : the official journal of the Society for Neuroscience 2002;22(20):8942-50.
2002: Cheryl L Wellington; Lisa M Ellerby; Claire-Anne Gutekunst; Danny Rogers; Simon Warby; Graham Kyrenia Rona; Odell Loubser; Jeremy M van Raamsdonk; Roshni R Singaraja; Yu-Zhou Yang; Juliette Gafni; Dale E Bredesen; Steven M Hersch; Blair R Leavitt; Sophie Roy; Donald W Nicholson; Michael R Hayden
Caspase cleavage of mutant huntingtin precedes neurodegeneration in Huntington's disease.
The Journal of neuroscience : the official journal of the Society for Neuroscience 2002;22(18):7862-72.
2002: Christopher A Meade; Yun-Ping Deng; Francesca R Fusco; Nobel Del Mar; Steven M Hersch; Dan Goldowitz; Anton Reiner
Cellular localization and development of neuronal intranuclear inclusions in striatal and cortical neurons in R6/2 transgenic mice.
The Journal of comparative neurology 2002;449(3):241-69.
2002: Anthone W Dunah; Hyunkyung Jeong; April Griffin; Yong-Man Kim; David G Standaert; Steven M Hersch; M Maral Mouradian; Anne B Young; Naoko Tanese; Dimitri Krainc
Sp1 and TAFII130 transcriptional activity disrupted in early Huntington's disease.
Science (New York, N.Y.) 2002;296(5576):2238-43.
2002: Vanessa C Wheeler; Claire-Anne Gutekunst; Vladimir Vrbanac; Lori-Anne Lebel; Gabriele Schilling; Steven M Hersch; Robert M Friedlander; James Gusella; Jean-Paul Vonsattel; David R Borchelt; Marcy E MacDonald
Early phenotypes that presage late-onset neurodegenerative disease allow testing of modifiers in Hdh CAG knock-in mice.
Human molecular genetics 2002;11(6):633-40.
2002: H Diana Rosas; Arthur K Liu; Steven M Hersch; Maureen Glessner; Robert J Ferrante; David H Salat; André J W van der Kouwe; Bruce G Jenkins; Anders Dale; Bruce Fischl
Regional and progressive thinning of the cortical ribbon in Huntington's disease.
Neurology 2002;58(5):695-701.
2002: Robert J Ferrante; Ole A Andreassen; Alpaslan Dedeoglu; Kimberly L Ferrante; Bruce G Jenkins; Steven M Hersch; M Flint Beal
Therapeutic effects of coenzyme Q10 and remacemide in transgenic mouse models of Huntington's disease.
The Journal of neuroscience : the official journal of the Society for Neuroscience 2002;22(5):1592-9.
2002: Tamara Kiechle; Alpaslan Dedeoglu; James K Kubilus; Neil W Kowall; M Flint Beal; Robert M Friedlander; Steven M Hersch; Robert J Ferrante
Cytochrome C and caspase-9 expression in Huntington's disease.
Neuromolecular medicine 2002;1(3):183-95.
2001: Steven M Hersch; H Diana Rosas
10 most commonly asked questions about Huntington's disease.
The neurologist 2001;7(6):364-8.
2001: Steven M Hersch; H Diana Rosas
Neuroprotective therapy for Huntington's disease: new prospects and challenges.
Expert review of neurotherapeutics 2001;1(1):111-8.
2001: A A Alcantara; L Mrzljak; RL Jakab; Allan Levey; Steven M Hersch; Patricia S Goldman-Rakic
Muscarinic m1 and m2 receptor proteins in local circuit and projection neurons of the primate striatum: anatomical evidence for cholinergic modulation of glutamatergic prefronto-striatal pathways.
The Journal of comparative neurology 2001;434(4):445-60.
2001: Ole A Andreassen; Alpaslan Dedeoglu; Robert J Ferrante; Bruce G Jenkins; Kimberly L Ferrante; M Thomas; Avi Friedlich; Susan E Browne; Gabriele Schilling; David R Borchelt; Steven M Hersch; Christopher A Ross; M Flint Beal
Creatine increase survival and delays motor symptoms in a transgenic animal model of Huntington's disease.
Neurobiology of disease 2001;8(3):479-91.
2001: Hong Yi; J Leunissen; G Shi; Claire-Anne Gutekunst; Steven M Hersch
A novel procedure for pre-embedding double immunogold-silver labeling at the ultrastructural level.
The journal of histochemistry and cytochemistry : official journal of the Histochemistry Society 2001;49(3):279-84.
2000: Thomas B Freeman; F Cicchetti; Robert A Hauser; TW Deacon; Xiao-Jiang Li; Steven M Hersch; G Michael Nauert; Paul Sanberg; Jeffrey Kordower; Samuel Saporta; Ole Isacson
Transplanted fetal striatum in Huntington's disease: phenotypic development and lack of pathology.
Proceedings of the National Academy of Sciences of the United States of America 2000;97(25):13877-82.
2000: T M Thomas; Yoland Smith; Allan Levey; Steven M Hersch
Cortical inputs to m2-immunoreactive striatal interneurons in rat and monkey.
Synapse (New York, N.Y.) 2000;37(4):252-61.
2000: Claire-Anne Gutekunst; Fran Norflus; Steven M Hersch
Recent advances in Huntington's disease.
Current opinion in neurology 2000;13(4):445-50.
2000: M Chen; Victor O Ona; Mingwei Li; Robert J Ferrante; Klaus B Fink; Shan Zhu; J Bian; L Guo; L A Farrell; Steven M Hersch; Wendy Hobbs; Jean-Paul Vonsattel; Jang-Ho Cha; Robert M Friedlander
Minocycline inhibits caspase-1 and caspase-3 expression and delays mortality in a transgenic mouse model of Huntington disease.
Nature medicine 2000;6(7):797-801.
2000: Robert J Ferrante; Ole A Andreassen; Bruce G Jenkins; Alpaslan Dedeoglu; S Kuemmerle; James K Kubilus; R Kaddurah-Daouk; Steven M Hersch; M Flint Beal
Neuroprotective effects of creatine in a transgenic mouse model of Huntington's disease.
The Journal of neuroscience : the official journal of the Society for Neuroscience 2000;20(12):4389-97.
2000: Vanessa C Wheeler; JK White; Claire-Anne Gutekunst; Vladimir Vrbanac; M Weaver; Xiao-Jiang Li; S H Li; H Yi; Jean-Paul Vonsattel; James Gusella; Steven M Hersch; Wojtek Auerbach; Alexandra L Joyner; Marcy E MacDonald
Long glutamine tracts cause nuclear localization of a novel form of huntingtin in medium spiny striatal neurons in HdhQ92 and HdhQ111 knock-in mice.
Human molecular genetics 2000;9(4):503-13.
1999: S Kuemmerle; Claire-Anne Gutekunst; Adam M Klein; Xiao-Jiang Li; Shi-Hua Li; M Flint Beal; Steven M Hersch; Robert J Ferrante
Huntington aggregates may not predict neuronal death in Huntington's disease.
Annals of neurology 1999;46(6):842-9.
1999: Abigail S Hackam; JG Hodgson; Roshni R Singaraja; T Zhang; Lu Gan; Claire-Anne Gutekunst; Steven M Hersch; Michael R Hayden
Evidence for both the nucleus and cytoplasm as subcellular sites of pathogenesis in Huntington's disease in cell culture and in transgenic mice expressing mutant huntingtin.
Philosophical transactions of the Royal Society of London. Series B, Biological sciences 1999;354(1386):1047-55.
1999: Annette K Taylor; Flora Tassone; P N Dyer; Steven M Hersch; Jennifer B Harris; William T Greenough; Randi J Hagerman
Tissue heterogeneity of the FMR1 mutation in a high-functioning male with fragile X syndrome.
American journal of medical genetics 1999;84(3):233-9.
1999: JG Hodgson; N Agopyan; Claire-Anne Gutekunst; Blair R Leavitt; F LePiane; Roshni R Singaraja; Desmond J Smith; Nagat Bissada; Krista McCutcheon; Jamal Nasir; Laure Jamot; Xiao-Jiang Li; M E Stevens; E Rosemond; John C Roder; A G Phillips; E M Rubin; Steven M Hersch; Michael R Hayden
A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration.
Neuron 1999;23(1):181-92.
1999: Claire-Anne Gutekunst; Shi-Hua Li; Hong Yi; J S Mulroy; S Kuemmerle; R Jones; D Rye; Robert J Ferrante; Steven M Hersch; Xiao-Jiang Li
Nuclear and neuropil aggregates in Huntington's disease: relationship to neuropathology.
The Journal of neuroscience : the official journal of the Society for Neuroscience 1999;19(7):2522-34.
1998: S H Li; Claire-Anne Gutekunst; Steven M Hersch; Xiao-Jiang Li
Association of HAP1 isoforms with a unique cytoplasmic structure.
Journal of neurochemistry 1998;71(5):2178-85.
1998: IA Klement; Pamela J Skinner; Michael D Kaytor; H Yi; Steven M Hersch; H Brent Clark; Huda Y Zoghbi; Harry T Orr
Ataxin-1 nuclear localization and aggregation: role in polyglutamine-induced disease in SCA1 transgenic mice.
Cell 1998;95(1):41-53.
1998: Claire-Anne Gutekunst; Shi-Hua Li; Hong Yi; Robert J Ferrante; Xiao-Jiang Li; Steven M Hersch
The cellular and subcellular localization of huntingtin-associated protein 1 (HAP1): comparison with huntingtin in rat and human.
The Journal of neuroscience : the official journal of the Society for Neuroscience 1998;18(19):7674-86.
1998: Shi-Hua Li; Seyed H Hosseini; Claire-Anne Gutekunst; Steven M Hersch; Robert J Ferrante; Xiao-Jiang Li
A human HAP1 homologue. Cloning, expression, and interaction with huntingtin.
The Journal of biological chemistry 1998;273(30):19220-7.
1998: Shi-Hua Li; Claire-Anne Gutekunst; Steven M Hersch; Xiao-Jiang Li
Interaction of huntingtin-associated protein with dynactin P150Glued.
The Journal of neuroscience : the official journal of the Society for Neuroscience 1998;18(4):1261-9.
1997: Jared M Ordway; Sara J Tallaksen-Greene; Claire-Anne Gutekunst; EM Bernstein; Jamie A Cearley; H W Wiener; Leon S Dure; R Lindsey; Steven M Hersch; Richard Scott Jope; Roger L Albin; Peter J Detloff
Ectopically expressed CAG repeats cause intranuclear inclusions and a progressive late onset neurological phenotype in the mouse.
Cell 1997;91(6):753-63.
1997: Steven M Hersch; Hong Yi; Craig J Heilman; Robert H Edwards; Allan Levey
Subcellular localization and molecular topology of the dopamine transporter in the striatum and substantia nigra.
The Journal of comparative neurology 1997;388(2):211-27.
1997: Robert J Ferrante; Claire-Anne Gutekunst; Francesca Persichetti; S M McNeil; Neil W Kowall; James Gusella; Marcy E MacDonald; M Flint Beal; Steven M Hersch
Heterogeneous topographic and cellular distribution of huntingtin expression in the normal human neostriatum.
The Journal of neuroscience : the official journal of the Society for Neuroscience 1997;17(9):3052-63.
1997: Yue Feng; Claire-Anne Gutekunst; D E Eberhart; Hong Yi; Stephen Warren; Steven M Hersch
Fragile X mental retardation protein: nucleocytoplasmic shuttling and association with somatodendritic ribosomes.
The Journal of neuroscience : the official journal of the Society for Neuroscience 1997;17(5):1539-47.
1996: Michelle L Gilmor; Norman R Nash; A Roghani; Robert H Edwards; Hong Yi; Steven M Hersch; Allan Levey
Expression of the putative vesicular acetylcholine transporter in rat brain and localization in cholinergic synaptic vesicles.
The Journal of neuroscience : the official journal of the Society for Neuroscience 1996;16(7):2179-90.
1996: SR Bradley; Allan Levey; Steven M Hersch; P Jeffrey Conn
Immunocytochemical localization of group III metabotropic glutamate receptors in the hippocampus with subtype-specific antibodies.
The Journal of neuroscience : the official journal of the Society for Neuroscience 1996;16(6):2044-56.
1995: Claire-Anne Gutekunst; Allan Levey; Craig J Heilman; W L Whaley; Hong Yi; Norman R Nash; Howard D Rees; J J Madden; Steven M Hersch
Identification and localization of huntingtin in brain and human lymphoblastoid cell lines with anti-fusion protein antibodies.
Proceedings of the National Academy of Sciences of the United States of America 1995;92(19):8710-4.
1995: Steven M Hersch; Brian Ciliax; Claire-Anne Gutekunst; Howard D Rees; Craig J Heilman; K K Yung; J Paul Bolam; E Ince; Hong Yi; Allan Levey
Electron microscopic analysis of D1 and D2 dopamine receptor proteins in the dorsal striatum and their synaptic relationships with motor corticostriatal afferents.
The Journal of neuroscience : the official journal of the Society for Neuroscience 1995;15(7 Pt 2):5222-37.
1995: K K Yung; J Paul Bolam; A David Smith; Steven M Hersch; Brian Ciliax; Allan Levey
Immunocytochemical localization of D1 and D2 dopamine receptors in the basal ganglia of the rat: light and electron microscopy.
Neuroscience 1995;65(3):709-30.
1995: Brian Ciliax; Craig J Heilman; L L Demchyshyn; Zdenek Pristupa; E Ince; Steven M Hersch; Hyman B Niznik; Allan Levey
The dopamine transporter: immunochemical characterization and localization in brain.
The Journal of neuroscience : the official journal of the Society for Neuroscience 1995;15(3 Pt 1):1714-23.
1995: Allan Levey; S M Edmunds; Steven M Hersch; R G Wiley; Craig J Heilman
Light and electron microscopic study of m2 muscarinic acetylcholine receptor in the basal forebrain of the rat.
The Journal of comparative neurology 1995;351(3):339-56.
1995: Steven M Hersch; Allan Levey
Diverse pre- and post-synaptic expression of m1-m4 muscarinic receptor proteins in neurons and afferents in the rat neostriatum.
Life sciences 1995;56(11-12):931-8.
1994: Steven M Hersch; R Jones; Walter J Koroshetz; K Quaid
The neurogenetics genie: testing for the Huntington's disease mutation.
Neurology 1994;44(8):1369-73.
1994: Steven M Hersch; Claire-Anne Gutekunst; Howard D Rees; Craig J Heilman; Allan Levey
Distribution of m1-m4 muscarinic receptor proteins in the rat striatum: light and electron microscopic immunocytochemistry using subtype-specific antibodies.
The Journal of neuroscience : the official journal of the Society for Neuroscience 1994;14(5 Pt 2):3351-63.
1993: Allan Levey; Steven M Hersch; David B Rye; Roger K Sunahara; Hyman B Niznik; C A Kitt; Donald L Price; R Maggio; Mark R Brann; Brian Ciliax
Localization of D1 and D2 dopamine receptors in brain with subtype-specific antibodies.
Proceedings of the National Academy of Sciences of the United States of America 1993;90(19):8861-5.
1992: R C Green; Felicia Goldstein; AP Auchus; R Presley; W S Clark; L Van Tuyl; J Green; Steven M Hersch; H R Karp
Treatment trial of oxiracetam in Alzheimer's disease.
Archives of neurology 1992;49(11):1135-6.
1990: S Kamei; Steven M Hersch; T Kurata; Y Takei
Coxsackie B antigen in the central nervous system of a patient with fatal acute encephalitis: immunohistochemical studies of formalin-fixed paraffin-embedded tissue.
Acta neuropathologica 1990;80(2):216-21.
1989: S Kamei; Steven M Hersch; T Takasu; Y Takei
[Acquired immunodeficiency syndrome presenting with neurological symptoms].
Nihon Naika Gakkai zasshi. The Journal of the Japanese Society of Internal Medicine 1989;78(3):426-7.
1985: P B Cipolloni; Steven M Hersch; E L White
The use of lectin transport in the mouse central nervous system as an anterograde axonal marker for electron microscopy.
Neuroscience letters 1985;58(1):43-7.
1984: E L White; G Benshalom; Steven M Hersch
Thalamocortical and other synapses involving nonspiny multipolar cells of mouse SmI cortex.
The Journal of comparative neurology 1984;229(3):311-20.
1982: Steven M Hersch; E L White
A quantitative study of the thalamocortical and other synapses in layer IV of pyramidal cells projecting from mouse SmI cortex to the caudate-putamen nucleus.
The Journal of comparative neurology 1982;211(3):217-25.
1982: E L White; Steven M Hersch
A quantitative study of thalamocortical and other synapses involving the apical dendrites of corticothalamic projection cells in mouse SmI cortex.
Journal of neurocytology 1982;11(1):137-57.
1981: Steven M Hersch; E L White
Thalamocortical synapses with corticothalamic projection neurons in mouse SmI cortex: electron microscopic demonstration of a monosynaptic feedback loop.
Neuroscience letters 1981;24(3):207-10.
1981: E L White; Steven M Hersch
Thalamocortical synapses of pyramidal cells which project from SmI to MsI cortex in the mouse.
The Journal of comparative neurology 1981;198(1):167-81.
1981: Steven M Hersch; E L White
Thalamocortical synapses involving identified neurons in mouse primary somatosensory cortex: a terminal degeneration and golgi/EM study.
The Journal of comparative neurology 1981;195(2):253-63.
1981: Steven M Hersch; E L White
Quantification of synapses formed with apical dendrites of Golgi-impregnated pyramidal cells: variability in thalamocortical inputs, but consistency in the ratios of asymmetrical to symmetrical synapses.
Neuroscience 1981;6(6):1043-51.
1980: E L White; Steven M Hersch; M P Rock
Synaptic sequences in mouse SmI cortex involving pyramidal cells labeled by retrograde filling with horseradish peroxidase.
Neuroscience letters 1980;19(2):149-54.