Joan S Steffan

Summary

Affiliation: University of California
Country: USA

Publications

  1. pmc Nicotinamide restores cognition in Alzheimer's disease transgenic mice via a mechanism involving sirtuin inhibition and selective reduction of Thr231-phosphotau
    Kim N Green
    Department of Neurobiology and Behavior, University of California, Irvine, Irvine, California 92697 4545, USA
    J Neurosci 28:11500-10. 2008
  2. pmc Does Huntingtin play a role in selective macroautophagy?
    Joan S Steffan
    Department of Psychiatry and Human Behavior, University of California, Irvine, CA, USA
    Cell Cycle 9:3401-13. 2010
  3. ncbi request reprint Targeting aggregation in the development of therapeutics for the treatment of Huntington's disease and other polyglutamine repeat diseases
    Joan S Steffan
    Department of Psychiatry and Human Behavior, University of California, Irvine, CA 92697 4260, USA
    Expert Opin Ther Targets 7:201-13. 2003
  4. ncbi request reprint The first 17 amino acids of Huntingtin modulate its sub-cellular localization, aggregation and effects on calcium homeostasis
    Erica Rockabrand
    Department of Psychiatry and Human Behavior, University of California, Gillespie 2121, Irvine, CA 92697, USA
    Hum Mol Genet 16:61-77. 2007
  5. pmc Phosphorylation of threonine 3: implications for Huntingtin aggregation and neurotoxicity
    Charity T Aiken
    Department of Developmental and Cell Biology, University of California, Irvine, California 92697, USA
    J Biol Chem 284:29427-36. 2009
  6. pmc IKK phosphorylates Huntingtin and targets it for degradation by the proteasome and lysosome
    Leslie Michels Thompson
    Department of Psychiatry and Human Behavior, University of California, Irvine, 92697, USA
    J Cell Biol 187:1083-99. 2009
  7. pmc Inhibition of specific HDACs and sirtuins suppresses pathogenesis in a Drosophila model of Huntington's disease
    Judit Pallos
    Department of Developmental and Cell Biology, University of California, Irvine, CA 92697, USA
    Hum Mol Genet 17:3767-75. 2008
  8. pmc Normal-repeat-length polyglutamine peptides accelerate aggregation nucleation and cytotoxicity of expanded polyglutamine proteins
    Natalia Slepko
    Department of Psychiatry and Human Behavior, University of California, Irvine, CA 92697 4260, USA
    Proc Natl Acad Sci U S A 103:14367-72. 2006
  9. ncbi request reprint SUMO modification of Huntingtin and Huntington's disease pathology
    Joan S Steffan
    Department of Psychiatry and Human Behavior, Gillespie 2121, University of California, Irvine, CA 92697, USA
    Science 304:100-4. 2004
  10. doi request reprint SUMO-2 and PIAS1 Modulate Insoluble Mutant Huntingtin Protein Accumulation
    Jacqueline Gire O'Rourke
    Department of Biological Chemistry, University of California, Irvine, Irvine, CA 92697, USA Department of Psychiatry and Human Behavior, University of California, Irvine, Irvine, CA 92697, USA Institute for Memory Impairments and Neurological Disorders, University of California, Irvine, Irvine, CA 92697, USA
    Cell Rep 4:362-75. 2013

Collaborators

Detail Information

Publications12

  1. pmc Nicotinamide restores cognition in Alzheimer's disease transgenic mice via a mechanism involving sirtuin inhibition and selective reduction of Thr231-phosphotau
    Kim N Green
    Department of Neurobiology and Behavior, University of California, Irvine, Irvine, California 92697 4545, USA
    J Neurosci 28:11500-10. 2008
    ..These preclinical findings suggest that oral nicotinamide may represent a safe treatment for AD and other tauopathies, and that phosphorylation of tau at Thr231 may regulate tau stability...
  2. pmc Does Huntingtin play a role in selective macroautophagy?
    Joan S Steffan
    Department of Psychiatry and Human Behavior, University of California, Irvine, CA, USA
    Cell Cycle 9:3401-13. 2010
    ..Pharmacologic activation of this pathway may be useful early in disease progression to treat HD and other neurodegenerative diseases characterized by the accumulation of disease proteins...
  3. ncbi request reprint Targeting aggregation in the development of therapeutics for the treatment of Huntington's disease and other polyglutamine repeat diseases
    Joan S Steffan
    Department of Psychiatry and Human Behavior, University of California, Irvine, CA 92697 4260, USA
    Expert Opin Ther Targets 7:201-13. 2003
    ..A discussion of the effect of antibodies, caspase inhibitors, chemical inhibitors, heat-shock proteins, suppressor peptides and transglutaminase inhibitors upon aggregation and disease is presented...
  4. ncbi request reprint The first 17 amino acids of Huntingtin modulate its sub-cellular localization, aggregation and effects on calcium homeostasis
    Erica Rockabrand
    Department of Psychiatry and Human Behavior, University of California, Gillespie 2121, Irvine, CA 92697, USA
    Hum Mol Genet 16:61-77. 2007
    ..Interestingly, neither mitochondrial nor ER associations seem to be required to promote long-term [Ca2+]i dyshomeostasis...
  5. pmc Phosphorylation of threonine 3: implications for Huntingtin aggregation and neurotoxicity
    Charity T Aiken
    Department of Developmental and Cell Biology, University of California, Irvine, California 92697, USA
    J Biol Chem 284:29427-36. 2009
    ..Thus, therapeutic strategies that modulate these events could in turn affect Htt pathogenesis...
  6. pmc IKK phosphorylates Huntingtin and targets it for degradation by the proteasome and lysosome
    Leslie Michels Thompson
    Department of Psychiatry and Human Behavior, University of California, Irvine, 92697, USA
    J Cell Biol 187:1083-99. 2009
    ..Thus, IKK activation may modulate mutant Htt neurotoxicity depending on the cell's ability to degrade the modified species...
  7. pmc Inhibition of specific HDACs and sirtuins suppresses pathogenesis in a Drosophila model of Huntington's disease
    Judit Pallos
    Department of Developmental and Cell Biology, University of California, Irvine, CA 92697, USA
    Hum Mol Genet 17:3767-75. 2008
    ..These results highlight a novel therapeutic approach for HD in the form of Sir2 inhibition and possible combinatorial inhibition of Sir2 and Rpd3...
  8. pmc Normal-repeat-length polyglutamine peptides accelerate aggregation nucleation and cytotoxicity of expanded polyglutamine proteins
    Natalia Slepko
    Department of Psychiatry and Human Behavior, University of California, Irvine, CA 92697 4260, USA
    Proc Natl Acad Sci U S A 103:14367-72. 2006
    ..The results suggest that the overall state of the polyGln protein network in a cellular environment may have a profound effect on the toxic consequences of polyGln expansion and thus may serve as a genetic modifier of age of onset in HD...
  9. ncbi request reprint SUMO modification of Huntingtin and Huntington's disease pathology
    Joan S Steffan
    Department of Psychiatry and Human Behavior, Gillespie 2121, University of California, Irvine, CA 92697, USA
    Science 304:100-4. 2004
    ..Lysine mutations that prevent both SUMOylation and ubiquitination of Httex1p reduce HD pathology, indicating that the contribution of SUMOylation to HD pathology extends beyond preventing Htt ubiquitination and degradation...
  10. doi request reprint SUMO-2 and PIAS1 Modulate Insoluble Mutant Huntingtin Protein Accumulation
    Jacqueline Gire O'Rourke
    Department of Biological Chemistry, University of California, Irvine, Irvine, CA 92697, USA Department of Psychiatry and Human Behavior, University of California, Irvine, Irvine, CA 92697, USA Institute for Memory Impairments and Neurological Disorders, University of California, Irvine, Irvine, CA 92697, USA
    Cell Rep 4:362-75. 2013
    ....
  11. ncbi request reprint A bivalent Huntingtin binding peptide suppresses polyglutamine aggregation and pathogenesis in Drosophila
    Aleksey Kazantsev
    Department of Biology, Center for Cancer Research, Massachusetts Institute of Technology, Cambridge, Massachusetts 02139, USA
    Nat Genet 30:367-76. 2002
    ..These results suggest that targeting the protein interactions leading to aggregate formation may be beneficial for the design and development of therapeutic agents for Huntington disease...
  12. pmc Suberoylanilide hydroxamic acid, a histone deacetylase inhibitor, ameliorates motor deficits in a mouse model of Huntington's disease
    Emma Hockly
    Medical and Molecular Genetics, Guy s, King s and St Thomas School of Medicine, King s College London, Eighth Floor Guy s Tower, Guy s Hospital, London SE1 9RT, United Kingdom
    Proc Natl Acad Sci U S A 100:2041-6. 2003
    ..SAHA dramatically improved the motor impairment in R6/2 mice, clearly validating the pursuit of this class of compounds as HD therapeutics...