Henry Paulson

Summary

Affiliation: University of Michigan
Country: USA

Publications

  1. pmc Genetics of dementia
    Henry L Paulson
    Department of Neurology, University of Michigan, Ann Arbor, Michigan 48109, USA
    Semin Neurol 31:449-60. 2011
  2. pmc Machado-Joseph disease/spinocerebellar ataxia type 3
    Henry Paulson
    Department of Neurology, University of Michigan, Ann Arbor, MI 48109 2200, USA
    Handb Clin Neurol 103:437-49. 2012
  3. pmc The spinocerebellar ataxias
    Henry L Paulson
    Department of Neurology, University of Michigan Health Systems, Ann Arbor, MI 48109, USA
    J Neuroophthalmol 29:227-37. 2009
  4. ncbi request reprint Poly-ubiquitin binding by the polyglutamine disease protein ataxin-3 links its normal function to protein surveillance pathways
    Yaohui Chai
    Department of Neurology, Carver College of Medicine, University of Iowa, Iowa City, Iowa 52242 1101, USA
    J Biol Chem 279:3605-11. 2004
  5. pmc X11alpha haploinsufficiency enhances Abeta amyloid deposition in Alzheimer's disease transgenic mice
    Inderjeet Saluja
    Department of Neurology, University of Michigan, USA
    Neurobiol Dis 36:162-8. 2009
  6. pmc Physiologic alterations in ataxia: channeling changes into novel therapies
    Vikram G Shakkottai
    Department of Neurology, University of Michigan, Ann Arbor, Michigan, USA
    Arch Neurol 66:1196-201. 2009
  7. pmc RNA-mediated neurodegeneration in repeat expansion disorders
    Peter K Todd
    Department of Neurology, University of Michigan, Ann Arbor, MI 48109, USA
    Ann Neurol 67:291-300. 2010
  8. pmc Activity and cellular functions of the deubiquitinating enzyme and polyglutamine disease protein ataxin-3 are regulated by ubiquitination at lysine 117
    Sokol V Todi
    Department of Neurology, University of Michigan Medical School, Ann Arbor, Michigan 48109, USA
    J Biol Chem 285:39303-13. 2010
  9. pmc Histone deacetylases suppress CGG repeat-induced neurodegeneration via transcriptional silencing in models of fragile X tremor ataxia syndrome
    Peter K Todd
    Department of Neurology, University of Michigan, Ann Arbor, Michigan, United States of America
    PLoS Genet 6:e1001240. 2010
  10. pmc In vivo suppression of polyglutamine neurotoxicity by C-terminus of Hsp70-interacting protein (CHIP) supports an aggregation model of pathogenesis
    AISLINN J WILLIAMS
    Graduate Program in Neuroscience and Medical Scientist Training Program, University of Iowa, 2206 MERF, Iowa City, IA 52242, USA
    Neurobiol Dis 33:342-53. 2009

Collaborators

Detail Information

Publications23

  1. pmc Genetics of dementia
    Henry L Paulson
    Department of Neurology, University of Michigan, Ann Arbor, Michigan 48109, USA
    Semin Neurol 31:449-60. 2011
    ..All major classes of dementia will be discussed but greatest attention will be given to the most common dementia, Alzheimer's disease, for which several new genetic factors were recently identified...
  2. pmc Machado-Joseph disease/spinocerebellar ataxia type 3
    Henry Paulson
    Department of Neurology, University of Michigan, Ann Arbor, MI 48109 2200, USA
    Handb Clin Neurol 103:437-49. 2012
    ..The specific properties of MJD/SCA3 and its disease protein are discussed in light of what is known about the entire class of polyglutamine diseases...
  3. pmc The spinocerebellar ataxias
    Henry L Paulson
    Department of Neurology, University of Michigan Health Systems, Ann Arbor, MI 48109, USA
    J Neuroophthalmol 29:227-37. 2009
    ..Although the SCAs are relentlessly progressive and currently untreatable, recent scientific advances have begun to shed light on various disease mechanisms that may lead to preventive therapies...
  4. ncbi request reprint Poly-ubiquitin binding by the polyglutamine disease protein ataxin-3 links its normal function to protein surveillance pathways
    Yaohui Chai
    Department of Neurology, Carver College of Medicine, University of Iowa, Iowa City, Iowa 52242 1101, USA
    J Biol Chem 279:3605-11. 2004
    ..Our results establish ataxin-3 as a poly-ubiquitin-binding protein, thereby linking its normal function to protein surveillance pathways already implicated in polyglutamine pathogenesis...
  5. pmc X11alpha haploinsufficiency enhances Abeta amyloid deposition in Alzheimer's disease transgenic mice
    Inderjeet Saluja
    Department of Neurology, University of Michigan, USA
    Neurobiol Dis 36:162-8. 2009
    ..The increased neuropathological indices of AD in mice expressing reduced X11alpha suggest a normal suppressor role for X11alpha on CNS Abeta/amyloid deposition...
  6. pmc Physiologic alterations in ataxia: channeling changes into novel therapies
    Vikram G Shakkottai
    Department of Neurology, University of Michigan, Ann Arbor, Michigan, USA
    Arch Neurol 66:1196-201. 2009
    ..Understanding these pathophysiologic changes may reveal novel therapeutic targets for symptomatic treatment of ataxia...
  7. pmc RNA-mediated neurodegeneration in repeat expansion disorders
    Peter K Todd
    Department of Neurology, University of Michigan, Ann Arbor, MI 48109, USA
    Ann Neurol 67:291-300. 2010
    ..Lastly, we comment on recent progress in therapeutic development for these RNA-dominant diseases...
  8. pmc Activity and cellular functions of the deubiquitinating enzyme and polyglutamine disease protein ataxin-3 are regulated by ubiquitination at lysine 117
    Sokol V Todi
    Department of Neurology, University of Michigan Medical School, Ann Arbor, Michigan 48109, USA
    J Biol Chem 285:39303-13. 2010
    ....
  9. pmc Histone deacetylases suppress CGG repeat-induced neurodegeneration via transcriptional silencing in models of fragile X tremor ataxia syndrome
    Peter K Todd
    Department of Neurology, University of Michigan, Ann Arbor, Michigan, United States of America
    PLoS Genet 6:e1001240. 2010
    ..Moreover, these results provide proof of principle that HAT inhibitors or HDAC activators might be used to selectively repress transcription at the FMR1 locus...
  10. pmc In vivo suppression of polyglutamine neurotoxicity by C-terminus of Hsp70-interacting protein (CHIP) supports an aggregation model of pathogenesis
    AISLINN J WILLIAMS
    Graduate Program in Neuroscience and Medical Scientist Training Program, University of Iowa, 2206 MERF, Iowa City, IA 52242, USA
    Neurobiol Dis 33:342-53. 2009
    ..Our results support an aggregation model of polyQ disease pathogenesis in which ataxin-3 microaggregates are a neurotoxic species, and suggest that enhancing CHIP activity is a possible route to therapy for SCA3 and other polyQ diseases...
  11. pmc The deubiquitinating enzyme ataxin-3, a polyglutamine disease protein, edits Lys63 linkages in mixed linkage ubiquitin chains
    Brett J Winborn
    Department of Neurology, University of Michigan, Ann Arbor, Michigan 48108, USA
    J Biol Chem 283:26436-43. 2008
    ..These findings establish ataxin-3 as a novel DUB that edits topologically complex chains...
  12. pmc Live-cell imaging reveals divergent intracellular dynamics of polyglutamine disease proteins and supports a sequestration model of pathogenesis
    Yaohui Chai
    Department of Neurology, 3160 Medical Labs, University of Iowa College of Medicine, Iowa City, IA 52242, USA
    Proc Natl Acad Sci U S A 99:9310-5. 2002
    ....
  13. ncbi request reprint CHIP suppresses polyglutamine aggregation and toxicity in vitro and in vivo
    Victor M Miller
    Department of Neurology, University of Iowa, Roy J and Lucille A Carver College of Medicine, Iowa City, Iowa 52242, USA
    J Neurosci 25:9152-61. 2005
    ..We conclude that CHIP is a critical mediator of the neuronal response to misfolded polyQ protein and represents a potential therapeutic target in this important class of neurodegenerative diseases...
  14. ncbi request reprint Cellular turnover of the polyglutamine disease protein ataxin-3 is regulated by its catalytic activity
    Sokol V Todi
    Department of Neurology, University of Iowa, Iowa City, Iowa 52242, USA
    J Biol Chem 282:29348-58. 2007
    ..Taken together, these and other findings suggest that the catalytic activity of this disease-linked deubiquitinating enzyme regulates several of its cellular properties, which in turn may influence disease pathogenesis...
  15. pmc Ubiquitination directly enhances activity of the deubiquitinating enzyme ataxin-3
    Sokol V Todi
    Department of Neurology, University of Michigan, Ann Arbor, MI 48109, USA
    EMBO J 28:372-82. 2009
    ..Ataxin-3 is the first reported DUB in which ubiquitination directly regulates catalytic activity. We propose a new function for protein ubiquitination in regulating the activity of certain DUBs and perhaps other enzymes...
  16. ncbi request reprint Dominantly inherited ataxias: lessons learned from Machado-Joseph disease/spinocerebellar ataxia type 3
    Henry L Paulson
    Department of Neurology, University of Iowa College of Medicine, Iowa City, Iowa 52242, USA
    Semin Neurol 27:133-42. 2007
    ..Also discussed are current and future therapeutic opportunities for MJD/SCA3 in particular, many of which have relevance to other SCAs...
  17. ncbi request reprint Aberrant cellular behavior of mutant torsinA implicates nuclear envelope dysfunction in DYT1 dystonia
    Pedro Gonzalez-Alegre
    Department of Neurology, Carver College of Medicine at The University of Iowa, Iowa City, Iowa 52242, USA
    J Neurosci 24:2593-601. 2004
    ..DYT1 dystonia can be added to the growing list of inherited neurological disorders involving the NE...
  18. pmc Conditional Niemann-Pick C mice demonstrate cell autonomous Purkinje cell neurodegeneration
    Matthew J Elrick
    Department of Pathology, University of Michigan, Ann Arbor, MI 48109 0605, USA
    Hum Mol Genet 19:837-47. 2010
    ..Our data establish that Npc1 deficiency leads to cell autonomous, selective neurodegeneration and suggest that the ataxic symptoms of NPC disease arise from Purkinje cell death rather than cellular dysfunction...
  19. ncbi request reprint RNA interference as potential therapy for neurodegenerative disease: applications to inclusion-body myositis?
    Henry Paulson
    Department of Neurology, Carver College of Medicine, University of Iowa, Iowa City, IA 52242, USA
    Neurology 66:S114-7. 2006
    ..Provided that proximal pathogenic targets are identified, RNAi could surface as a potential therapeutic strategy to modulate their expression...
  20. pmc Allele-specific silencing of dominant disease genes
    Victor M Miller
    Department of Neurology, Graduate Program in Genetics, University of Iowa Roy J and Lucille A Carver College of Medicine, Iowa City, IA 52242, USA
    Proc Natl Acad Sci U S A 100:7195-200. 2003
    ..These studies establish that siRNA can be engineered to silence disease genes differing by a single nucleotide and highlight a key role for SNPs in extending the utility of siRNA in dominantly inherited disorders...
  21. ncbi request reprint RNA interference in neuroscience: progress and challenges
    Victor M Miller
    Department of Neurology, University of Iowa Roy J and Lucille A Carver College of Medicine, Iowa City, IA 52242 1101, USA
    Cell Mol Neurobiol 25:1195-207. 2005
    ..We then describe the methods developed to manipulate RNAi in the laboratory and its applications to neuroscience. Finally, we focus on the potential therapeutic application of RNAi to neurological disease...
  22. pmc Allele-specific RNAi mitigates phenotypic progression in a transgenic model of Alzheimer's disease
    Edgardo Rodriguez-Lebron
    Department of Neurology, University of Michigan, Ann Arbor, Michigan, USA
    Mol Ther 17:1563-73. 2009
    ..Our results support the development of allele-specific RNAi strategies to treat familial AD and other dominantly inherited neurodegenerative diseases...
  23. ncbi request reprint Silencing primary dystonia: lentiviral-mediated RNA interference therapy for DYT1 dystonia
    Pedro Gonzalez-Alegre
    Department of Neurology, Carver College of Medicine, University of Iowa, Iowa City, Iowa 52242, USA
    J Neurosci 25:10502-9. 2005
    ..These results support the potential use of viral-mediated RNAi as a therapy for DYT1 dystonia and establish the basis for preclinical testing in animal models of the disease...

Research Grants16

  1. Mechanisms of Polyglutamine Neurodegeneration
    Henry L Paulson; Fiscal Year: 2010
    ..This grant will explore the basis of SCA3 in order that we can ultimately develop therapies for this fatal disease based on a better understanding of disease mechanisms. ..
  2. Integrating Quality Control: Studies of CHIP in Age-related Neurodegeneration
    Henry Paulson; Fiscal Year: 2009
    ..Understanding this machinery may suggest routes to therapy for a large range of sporadic and hereditary neurodegenerative diseases that occur as we age. ..
  3. Mechanisms of Polyglutamine Neurodegeneration
    Henry Paulson; Fiscal Year: 2009
    ..They may also shed light on the role of protein quality control, and particularly of de-ubiquitinating enzymes, in age-related neurodegenerative diseases characterized by protein misfolding and aggregation. ..
  4. Analysis of Fbx2 Family of Ubiquitin Ligases
    Henry Paulson; Fiscal Year: 2007
    ..The results may also have implications for the pathogenesis of two poorly understood neurological diseases, DYT1 dystonia and familial neuroserpin dementia. ..
  5. Mechanisms of Polyglutamine Neurodegeneration
    Henry Paulson; Fiscal Year: 2007
    ..They may also shed light on the role of protein quality control, and particularly of de-ubiquitinating enzymes, in age-related neurodegenerative diseases characterized by protein misfolding and aggregation. ..
  6. MECHANISMS OF GLUTAMINE REPEAT NEURODEGENERATION
    Henry Paulson; Fiscal Year: 2004
    ..abstract_text> ..
  7. Integrating Quality Control: Studies of CHIP in Age-related Neurodegeneration
    Henry L Paulson; Fiscal Year: 2010
    ..Understanding this machinery may suggest routes to therapy for a large range of sporadic and hereditary neurodegenerative diseases that occur as we age. ..