James Shorter Department of Biochemistry and Biophysics Perelman School of Medicine at the University of Pennsylvania Biography Publications Institution JoVE Articles James Shorter has not added a biography. If you are James Shorter and would like to personalize this page please email our Author Liaison for assistance. Publications Counteracting Semen-mediated Enhancement of HIV Infection and Enveloped Virus Infection by a Lysine-specific Molecular Tweezer AIDS Research and Human Retroviruses. Oct, 2014 | Pubmed ID: 25357741 Suramin Inhibits Hsp104 ATPase and Disaggregase Activity PloS One. 2014 | Pubmed ID: 25299406 Specific Aromatic Foldamers Potently Inhibit Spontaneous and Seeded Aβ42 and Aβ43 Fibril Assembly The Biochemical Journal. Nov, 2014 | Pubmed ID: 25142005 Potentiated Hsp104 Variants Suppress Toxicity of Diverse Neurodegenerative Disease-linked Proteins Disease Models & Mechanisms. Oct, 2014 | Pubmed ID: 25062688 A Cellular System That Degrades Misfolded Proteins and Protects Against Neurodegeneration Molecular Cell. Jul, 2014 | Pubmed ID: 24882209 Reversing Deleterious Protein Aggregation with Re-engineered Protein Disaggregases Cell Cycle (Georgetown, Tex.). May, 2014 | Pubmed ID: 24694655 ALS-associated Mutation FUS-R521C Causes DNA Damage and RNA Splicing Defects The Journal of Clinical Investigation. Mar, 2014 | Pubmed ID: 24509083 Potentiated Hsp104 Variants Antagonize Diverse Proteotoxic Misfolding Events Cell. Jan, 2014 | Pubmed ID: 24439375 Conserved Distal Loop Residues in the Hsp104 and ClpB Middle Domain Contact Nucleotide-binding Domain 2 and Enable Hsp70-dependent Protein Disaggregation The Journal of Biological Chemistry. Jan, 2014 | Pubmed ID: 24280225 Disease Mutations in the Prion-like Domains of HnRNPA1 and HnRNPA2/B1 Introduce Potent Steric Zippers That Drive Excess RNP Granule Assembly Rare Diseases (Austin, Tex.). 2013 | Pubmed ID: 25002999 The Metazoan Protein Disaggregase and Amyloid Depolymerase System: Hsp110, Hsp70, Hsp40, and Small Heat Shock Proteins Prion. Nov-Dec, 2013 | Pubmed ID: 24401655 Hsp104 Suppresses Polyglutamine-induced Degeneration Post Onset in a Drosophila MJD/SCA3 Model PLoS Genetics. 2013 | Pubmed ID: 24039611 Fission Yeast Does Not Age Under Favorable Conditions, but Does So After Stress Current Biology : CB. Oct, 2013 | Pubmed ID: 24035542 Stress Granules As Crucibles of ALS Pathogenesis The Journal of Cell Biology. Apr, 2013 | Pubmed ID: 23629963 Mutations in Prion-like Domains in HnRNPA2B1 and HnRNPA1 Cause Multisystem Proteinopathy and ALS Nature. Mar, 2013 | Pubmed ID: 23455423 The Surprising Role of Amyloid Fibrils in HIV Infection Biology. 2012 | Pubmed ID: 24832047 Hsp104 Drives "protein-only" Positive Selection of Sup35 Prion Strains Encoding Strong [PSI(+)] Chemistry & Biology. Nov, 2012 | Pubmed ID: 23177195 Operational Plasticity Enables Hsp104 to Disaggregate Diverse Amyloid and Nonamyloid Clients Cell. Nov, 2012 | Pubmed ID: 23141537 Inhibition of RNA Lariat Debranching Enzyme Suppresses TDP-43 Toxicity in ALS Disease Models Nature Genetics. Dec, 2012 | Pubmed ID: 23104007 RNA-Binding Proteins in Amyotrophic Lateral Sclerosis and Neurodegeneration Neurology Research International. 2012 | Pubmed ID: 22919483 Small Heat Shock Proteins Potentiate Amyloid Dissolution by Protein Disaggregases from Yeast and Humans PLoS Biology. 2012 | Pubmed ID: 22723742 Evaluating the Role of the FUS/TLS-related Gene EWSR1 in Amyotrophic Lateral Sclerosis Human Molecular Genetics. Apr, 2012 | Pubmed ID: 22454397 The Tip of the Iceberg: RNA-binding Proteins with Prion-like Domains in Neurodegenerative Disease Brain Research. Jun, 2012 | Pubmed ID: 22445064 The Elusive Middle Domain of Hsp104 and ClpB: Location and Function Biochimica Et Biophysica Acta. Jan, 2012 | Pubmed ID: 21843558 A Yeast Functional Screen Predicts New Candidate ALS Disease Genes Proceedings of the National Academy of Sciences of the United States of America. Dec, 2011 | Pubmed ID: 22065782 The Mammalian Disaggregase Machinery: Hsp110 Synergizes with Hsp70 and Hsp40 to Catalyze Protein Disaggregation and Reactivation in a Cell-free System PloS One. 2011 | Pubmed ID: 22022600 RNA-binding Proteins with Prion-like Domains in ALS and FTLD-U Prion. Jul, 2011 | Pubmed ID: 21847013 Shock and Awe: Unleashing the Heat Shock Response to Treat Huntington Disease The Journal of Clinical Investigation. Aug, 2011 | Pubmed ID: 21785212 Molecular Determinants and Genetic Modifiers of Aggregation and Toxicity for the ALS Disease Protein FUS/TLS PLoS Biology. Apr, 2011 | Pubmed ID: 21541367 Countering Amyloid Polymorphism and Drug Resistance with Minimal Drug Cocktails Prion. Oct-Dec, 2010 | Pubmed ID: 20935457 Emergence and Natural Selection of Drug-resistant Prions Molecular BioSystems. Jul, 2010 | Pubmed ID: 20422111 Prion-like Disorders: Blurring the Divide Between Transmissibility and Infectivity Journal of Cell Science. Apr, 2010 | Pubmed ID: 20356930 Applying Hsp104 to Protein-misfolding Disorders Biochemistry and Cell Biology = Biochimie Et Biologie Cellulaire. Feb, 2010 | Pubmed ID: 20130674 A Synergistic Small-molecule Combination Directly Eradicates Diverse Prion Strain Structures Nature Chemical Biology. Dec, 2009 | Pubmed ID: 19915541 N-terminal Domains Elicit Formation of Functional Pmel17 Amyloid Fibrils The Journal of Biological Chemistry. Dec, 2009 | Pubmed ID: 19840945 TDP-43 is Intrinsically Aggregation-prone, and Amyotrophic Lateral Sclerosis-linked Mutations Accelerate Aggregation and Increase Toxicity The Journal of Biological Chemistry. Jul, 2009 | Pubmed ID: 19465477 Motor Mechanism for Protein Threading Through Hsp104 Molecular Cell. Apr, 2009 | Pubmed ID: 19362537 Prion Proteostasis: Hsp104 Meets Its Supporting Cast Prion. Oct-Dec, 2008 | Pubmed ID: 19242125 The Mad2 Partial Unfolding Model: Regulating Mitosis Through Mad2 Conformational Switching The Journal of Cell Biology. Dec, 2008 | Pubmed ID: 19029339 Hsp104, Hsp70 and Hsp40 Interplay Regulates Formation, Growth and Elimination of Sup35 Prions The EMBO Journal. Oct, 2008 | Pubmed ID: 18833196 A PDZ-binding Motif Controls Basolateral Targeting of Syndecan-1 Along the Biosynthetic Pathway in Polarized Epithelial Cells Traffic (Copenhagen, Denmark). Nov, 2008 | Pubmed ID: 18764819 Hsp104 Antagonizes Alpha-synuclein Aggregation and Reduces Dopaminergic Degeneration in a Rat Model of Parkinson Disease The Journal of Clinical Investigation. Sep, 2008 | Pubmed ID: 18704197 Escaping Amyloid Fate Nature Structural & Molecular Biology. Jun, 2008 | Pubmed ID: 18523464 Direct and Selective Elimination of Specific Prions and Amyloids by 4,5-dianilinophthalimide and Analogs Proceedings of the National Academy of Sciences of the United States of America. May, 2008 | Pubmed ID: 18480256 Hsp110 Chaperones Regulate Prion Formation and Propagation in S. Cerevisiae by Two Discrete Activities PloS One. 2008 | Pubmed ID: 18335038 The Parkinson's Disease Protein Alpha-synuclein Disrupts Cellular Rab Homeostasis Proceedings of the National Academy of Sciences of the United States of America. Jan, 2008 | Pubmed ID: 18162536 Hsp104: a Weapon to Combat Diverse Neurodegenerative Disorders Neuro-Signals. 2008 | Pubmed ID: 18097161 Atypical AAA+ Subunit Packing Creates an Expanded Cavity for Disaggregation by the Protein-remodeling Factor Hsp104 Cell. Dec, 2007 | Pubmed ID: 18160044 Prime Time for Alpha-synuclein The Journal of Neuroscience : the Official Journal of the Society for Neuroscience. Mar, 2007 | Pubmed ID: 17344380 Asymmetric Deceleration of ClpB or Hsp104 ATPase Activity Unleashes Protein-remodeling Activity Nature Structural & Molecular Biology. Feb, 2007 | Pubmed ID: 17259993 Destruction or Potentiation of Different Prions Catalyzed by Similar Hsp104 Remodeling Activities Molecular Cell. Aug, 2006 | Pubmed ID: 16885031 Prions As Adaptive Conduits of Memory and Inheritance Nature Reviews. Genetics. Jun, 2005 | Pubmed ID: 15931169 A Cryptic Rab1-binding Site in the P115 Tethering Protein The Journal of Biological Chemistry. Jul, 2005 | Pubmed ID: 15878873 Navigating the ClpB Channel to Solution Nature Structural & Molecular Biology. Jan, 2005 | Pubmed ID: 15689967 Hsp104 Catalyzes Formation and Elimination of Self-replicating Sup35 Prion Conformers Science (New York, N.Y.). Jun, 2004 | Pubmed ID: 15155912 A Direct Role for GRASP65 As a Mitotically Regulated Golgi Stacking Factor The EMBO Journal. Jul, 2003 | Pubmed ID: 12839990 Golgi Architecture and Inheritance Annual Review of Cell and Developmental Biology. 2002 | Pubmed ID: 12142281 Sequential SNARE Disassembly and GATE-16-GOS-28 Complex Assembly Mediated by Distinct NSF Activities Drives Golgi Membrane Fusion The Journal of Cell Biology. Jun, 2002 | Pubmed ID: 12070132 Sequential Tethering of Golgins and Catalysis of SNAREpin Assembly by the Vesicle-tethering Protein P115 The Journal of Cell Biology. Apr, 2002 | Pubmed ID: 11927603 Isolere forsterkes Hsp104 Varianter Bruke gjær Proteinopathy modeller Meredith E. Jackrel1, Amber Tariq1, Keolamau Yee1, Rachel Weitzman1, James Shorter1 1Department of Biochemistry and Biophysics, Perelman School of Medicine at the University of Pennsylvania Biologie Purification of Hsp104, a Protein Disaggregase JoVE 3190 Biologie
Isolere forsterkes Hsp104 Varianter Bruke gjær Proteinopathy modeller Meredith E. Jackrel1, Amber Tariq1, Keolamau Yee1, Rachel Weitzman1, James Shorter1 1Department of Biochemistry and Biophysics, Perelman School of Medicine at the University of Pennsylvania Biologie