Aaron Gitler

Summary

Affiliation: University of Pennsylvania
Country: USA

Publications

  1. pmc Ataxin-2 intermediate-length polyglutamine expansions are associated with increased risk for ALS
    Andrew C Elden
    Department of Cell and Developmental Biology, University of Pennsylvania, Philadelphia, Pennsylvania 19104, USA
    Nature 466:1069-75. 2010
  2. pmc Molecular determinants and genetic modifiers of aggregation and toxicity for the ALS disease protein FUS/TLS
    Zhihui Sun
    Department of Cell and Developmental Biology, The University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, United States of America
    PLoS Biol 9:e1000614. 2011
  3. pmc PolyQ repeat expansions in ATXN2 associated with ALS are CAA interrupted repeats
    Zhenming Yu
    Department of Biology, University of Pennsylvania, Philadelphia, Pennsylvania, United States of America
    PLoS ONE 6:e17951. 2011
  4. pmc RNA-binding proteins with prion-like domains in ALS and FTLD-U
    Aaron D Gitler
    Department of Cell and Developmental Biology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA
    Prion 5:179-87. 2011
  5. pmc Model organisms reveal insight into human neurodegenerative disease: ataxin-2 intermediate-length polyglutamine expansions are a risk factor for ALS
    Nancy M Bonini
    Department of Biology, University of Pennsylvania, Philadelphia, PA 19104, USA
    J Mol Neurosci 45:676-83. 2011
  6. ncbi request reprint Beer and bread to brains and beyond: can yeast cells teach us about neurodegenerative disease?
    Aaron D Gitler
    Department of Cell and Developmental Biology, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    Neurosignals 16:52-62. 2008
  7. pmc TDP-43 is intrinsically aggregation-prone, and amyotrophic lateral sclerosis-linked mutations accelerate aggregation and increase toxicity
    Brian S Johnson
    Department of Cell and Developmental Biology, The University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA
    J Biol Chem 284:20329-39. 2009
  8. pmc Evaluating the prevalence of polyglutamine repeat expansions in amyotrophic lateral sclerosis
    T Lee
    Department of Cell and Developmental Biology, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    Neurology 76:2062-5. 2011
  9. pmc A yeast TDP-43 proteinopathy model: Exploring the molecular determinants of TDP-43 aggregation and cellular toxicity
    Brian S Johnson
    Department of Cell and Developmental Biology, University of Pennsylvania, Philadelphia, PA 19104, USA
    Proc Natl Acad Sci U S A 105:6439-44. 2008
  10. pmc Cardiac hypertrophy and histone deacetylase-dependent transcriptional repression mediated by the atypical homeodomain protein Hop
    Hyun Kook
    Cardiovascular Division, University of Pennsylvania Health System, Philadelphia, Pennsylvania, USA
    J Clin Invest 112:863-71. 2003

Collaborators

Detail Information

Publications13

  1. pmc Ataxin-2 intermediate-length polyglutamine expansions are associated with increased risk for ALS
    Andrew C Elden
    Department of Cell and Developmental Biology, University of Pennsylvania, Philadelphia, Pennsylvania 19104, USA
    Nature 466:1069-75. 2010
    ..Furthermore, these findings indicate that the TDP-43-ATXN2 interaction may be a promising target for therapeutic intervention in ALS and other TDP-43 proteinopathies...
  2. pmc Molecular determinants and genetic modifiers of aggregation and toxicity for the ALS disease protein FUS/TLS
    Zhihui Sun
    Department of Cell and Developmental Biology, The University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, United States of America
    PLoS Biol 9:e1000614. 2011
    ..Our findings suggest that TDP-43 and FUS, though similar RNA-binding proteins, aggregate and confer disease phenotypes via distinct mechanisms. These differences will likely have important therapeutic implications...
  3. pmc PolyQ repeat expansions in ATXN2 associated with ALS are CAA interrupted repeats
    Zhenming Yu
    Department of Biology, University of Pennsylvania, Philadelphia, Pennsylvania, United States of America
    PLoS ONE 6:e17951. 2011
    ....
  4. pmc RNA-binding proteins with prion-like domains in ALS and FTLD-U
    Aaron D Gitler
    Department of Cell and Developmental Biology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA
    Prion 5:179-87. 2011
    ..Collectively, however, our findings lead us to suggest that FUS and TDP-43, though similar RNA-binding proteins, likely aggregate and confer disease phenotypes via distinct mechanisms...
  5. pmc Model organisms reveal insight into human neurodegenerative disease: ataxin-2 intermediate-length polyglutamine expansions are a risk factor for ALS
    Nancy M Bonini
    Department of Biology, University of Pennsylvania, Philadelphia, PA 19104, USA
    J Mol Neurosci 45:676-83. 2011
    ..These studies provide an example of how model systems, when extended to human cells and human patient tissue, can reveal new mechanistic insight into disease...
  6. ncbi request reprint Beer and bread to brains and beyond: can yeast cells teach us about neurodegenerative disease?
    Aaron D Gitler
    Department of Cell and Developmental Biology, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    Neurosignals 16:52-62. 2008
    ..In this review, I highlight recent work investigating mechanisms of cellular toxicity in a yeast Parkinson's disease model and discuss how similar approaches are being applied to additional neurodegenerative diseases...
  7. pmc TDP-43 is intrinsically aggregation-prone, and amyotrophic lateral sclerosis-linked mutations accelerate aggregation and increase toxicity
    Brian S Johnson
    Department of Cell and Developmental Biology, The University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA
    J Biol Chem 284:20329-39. 2009
    ..Thus, TDP-43 is intrinsically aggregation-prone, and its propensity for toxic misfolding trajectories is accentuated by specific ALS-linked mutations...
  8. pmc Evaluating the prevalence of polyglutamine repeat expansions in amyotrophic lateral sclerosis
    T Lee
    Department of Cell and Developmental Biology, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    Neurology 76:2062-5. 2011
    ....
  9. pmc A yeast TDP-43 proteinopathy model: Exploring the molecular determinants of TDP-43 aggregation and cellular toxicity
    Brian S Johnson
    Department of Cell and Developmental Biology, University of Pennsylvania, Philadelphia, PA 19104, USA
    Proc Natl Acad Sci U S A 105:6439-44. 2008
    ..This work provides a mechanistic framework for investigating the toxicity of TDP-43 aggregation relevant to human disease and establishes a manipulable, high-throughput model for discovering potential therapeutic strategies...
  10. pmc Cardiac hypertrophy and histone deacetylase-dependent transcriptional repression mediated by the atypical homeodomain protein Hop
    Hyun Kook
    Cardiovascular Division, University of Pennsylvania Health System, Philadelphia, Pennsylvania, USA
    J Clin Invest 112:863-71. 2003
    ..Thus, chromatin remodeling and repression of otherwise active transcriptional processes can result in hypertrophy and heart failure, and this process can be blocked with chemical HDAC inhibitors...
  11. pmc The Parkinson's disease protein alpha-synuclein disrupts cellular Rab homeostasis
    Aaron D Gitler
    Whitehead Institute for Biomedical Research and Howard Hughes Medical Institute, Cambridge, MA 02142, USA
    Proc Natl Acad Sci U S A 105:145-50. 2008
    ..Thus, alpha-syn causes general defects in vesicle trafficking, to which dopaminergic neurons are especially sensitive...
  12. pmc A suite of Gateway cloning vectors for high-throughput genetic analysis in Saccharomyces cerevisiae
    Simon Alberti
    Whitehead Institute for Biomedical Research, Cambridge, MA, USA
    Yeast 24:913-9. 2007
    ..We have made this yeast Gateway vector kit available to the research community via the non-profit Addgene Plasmid Repository (http://www.addgene.org/yeast_gateway)...
  13. pmc Alpha-synuclein blocks ER-Golgi traffic and Rab1 rescues neuron loss in Parkinson's models
    Antony A Cooper
    School of Biological Sciences, University of Missouri Kansas City, Kansas City, MO 64110, USA
    Science 313:324-8. 2006
    ..Thus, synucleinopathies may result from disruptions in basic cellular functions that interface with the unique biology of particular neurons to make them especially vulnerable...

Research Grants2

  1. Molecular basis of TDP-43 proteinopathies: disease models and mechanisms
    Aaron Gitler; Fiscal Year: 2009
    ..elegans) to explore TDP-43 disease mechanisms and validating modifier genes from our genetic screens. ..
  2. Molecular basis of TDP-43 proteinopathies: disease models and mechanisms
    Aaron D Gitler; Fiscal Year: 2010
    ..elegans) to explore TDP-43 disease mechanisms and validating modifier genes from our genetic screens. ..