James Shorter

Department of Biochemistry and Biophysics, University of Pennsylvania

Affiliated withUniversity of Pennsylvania Perelman School of Medicine at the University of Pennsylvania

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JoVE Journal Publications

ArticleTotal : 2	Year
Publication title Cited by 24	2011
Publication title Cited by 16	2014

Other Publications

Article	Year
Sequential tethering of Golgins and catalysis of SNAREpin assembly by the vesicle-tethering protein p115. The Journal of cell biology\| PubMed ID: 11927603	2002
Sequential SNARE disassembly and GATE-16-GOS-28 complex assembly mediated by distinct NSF activities drives Golgi membrane fusion. The Journal of cell biology\| PubMed ID: 12070132	2002
Golgi architecture and inheritance. Annual review of cell and developmental biology\| PubMed ID: 12142281	2002
A direct role for GRASP65 as a mitotically regulated Golgi stacking factor. The EMBO journal\| PubMed ID: 12839990	2003
Hsp104 catalyzes formation and elimination of self-replicating Sup35 prion conformers. Science (New York, N.Y.)\| PubMed ID: 15155912	2004
Navigating the ClpB channel to solution. Nature structural & molecular biology\| PubMed ID: 15689967	2005
A cryptic Rab1-binding site in the p115 tethering protein. The Journal of biological chemistry\| PubMed ID: 15878873	2005
Prions as adaptive conduits of memory and inheritance. Nature reviews. Genetics\| PubMed ID: 15931169	2005
Destruction or potentiation of different prions catalyzed by similar Hsp104 remodeling activities. Molecular cell\| PubMed ID: 16885031	2006
Asymmetric deceleration of ClpB or Hsp104 ATPase activity unleashes protein-remodeling activity. Nature structural & molecular biology\| PubMed ID: 17259993	2007
Prime time for alpha-synuclein. The Journal of neuroscience : the official journal of the Society for Neuroscience\| PubMed ID: 17344380	2007
Hsp104: a weapon to combat diverse neurodegenerative disorders. Neuro-Signals\| PubMed ID: 18097161	2008
Atypical AAA+ subunit packing creates an expanded cavity for disaggregation by the protein-remodeling factor Hsp104. Cell\| PubMed ID: 18160044	2007
The Parkinson's disease protein alpha-synuclein disrupts cellular Rab homeostasis. Proceedings of the National Academy of Sciences of the United States of America\| PubMed ID: 18162536	2008
Hsp110 chaperones regulate prion formation and propagation in S. cerevisiae by two discrete activities. PloS one\| PubMed ID: 18335038	2008
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\| PubMed ID: 18480256	2008
Escaping amyloid fate. Nature structural & molecular biology\| PubMed ID: 18523464	2008
Hsp104 antagonizes alpha-synuclein aggregation and reduces dopaminergic degeneration in a rat model of Parkinson disease. The Journal of clinical investigation\| PubMed ID: 18704197	2008
A PDZ-binding motif controls basolateral targeting of syndecan-1 along the biosynthetic pathway in polarized epithelial cells. Traffic (Copenhagen, Denmark)\| PubMed ID: 18764819	2008
Hsp104, Hsp70 and Hsp40 interplay regulates formation, growth and elimination of Sup35 prions. The EMBO journal\| PubMed ID: 18833196	2008
The Mad2 partial unfolding model: regulating mitosis through Mad2 conformational switching. The Journal of cell biology\| PubMed ID: 19029339	2008
Prion proteostasis: Hsp104 meets its supporting cast. Prion\| PubMed ID: 19242125	2008
Motor mechanism for protein threading through Hsp104. Molecular cell\| PubMed ID: 19362537	2009
TDP-43 is intrinsically aggregation-prone, and amyotrophic lateral sclerosis-linked mutations accelerate aggregation and increase toxicity. The Journal of biological chemistry\| PubMed ID: 19465477	2009
N-terminal domains elicit formation of functional Pmel17 amyloid fibrils. The Journal of biological chemistry\| PubMed ID: 19840945	2009
A synergistic small-molecule combination directly eradicates diverse prion strain structures. Nature chemical biology\| PubMed ID: 19915541	2009
Applying Hsp104 to protein-misfolding disorders. Biochemistry and cell biology = Biochimie et biologie cellulaire\| PubMed ID: 20130674	2010
Prion-like disorders: blurring the divide between transmissibility and infectivity. Journal of cell science\| PubMed ID: 20356930	2010
Emergence and natural selection of drug-resistant prions. Molecular bioSystems\| PubMed ID: 20422111	2010
Countering amyloid polymorphism and drug resistance with minimal drug cocktails. Prion\| PubMed ID: 20935457	2010
Molecular determinants and genetic modifiers of aggregation and toxicity for the ALS disease protein FUS/TLS. PLoS biology\| PubMed ID: 21541367	2011
Shock and awe: unleashing the heat shock response to treat Huntington disease. The Journal of clinical investigation\| PubMed ID: 21785212	2011
The elusive middle domain of Hsp104 and ClpB: Location and function. Biochimica et biophysica acta\| PubMed ID: 21843558	2012
RNA-binding proteins with prion-like domains in ALS and FTLD-U. Prion\| PubMed ID: 21847013	2011
The mammalian disaggregase machinery: Hsp110 synergizes with Hsp70 and Hsp40 to catalyze protein disaggregation and reactivation in a cell-free system. PloS one\| PubMed ID: 22022600	2011
A yeast functional screen predicts new candidate ALS disease genes. Proceedings of the National Academy of Sciences of the United States of America\| PubMed ID: 22065782	2011
The tip of the iceberg: RNA-binding proteins with prion-like domains in neurodegenerative disease. Brain research\| PubMed ID: 22445064	2012
Evaluating the role of the FUS/TLS-related gene EWSR1 in amyotrophic lateral sclerosis. Human molecular genetics\| PubMed ID: 22454397	2012
Small heat shock proteins potentiate amyloid dissolution by protein disaggregases from yeast and humans. PLoS biology\| PubMed ID: 22723742	2012
RNA-Binding Proteins in Amyotrophic Lateral Sclerosis and Neurodegeneration. Neurology research international\| PubMed ID: 22919483	2012
Inhibition of RNA lariat debranching enzyme suppresses TDP-43 toxicity in ALS disease models. Nature genetics\| PubMed ID: 23104007	2012
Operational plasticity enables hsp104 to disaggregate diverse amyloid and nonamyloid clients. Cell\| PubMed ID: 23141537	2012
Hsp104 drives "protein-only" positive selection of Sup35 prion strains encoding strong [PSI(+)]. Chemistry & biology\| PubMed ID: 23177195	2012
Mutations in prion-like domains in hnRNPA2B1 and hnRNPA1 cause multisystem proteinopathy and ALS. Nature\| PubMed ID: 23455423	2013
Stress granules as crucibles of ALS pathogenesis. The Journal of cell biology\| PubMed ID: 23629963	2013
Fission yeast does not age under favorable conditions, but does so after stress. Current biology : CB\| PubMed ID: 24035542	2013
Hsp104 suppresses polyglutamine-induced degeneration post onset in a drosophila MJD/SCA3 model. PLoS genetics\| PubMed ID: 24039611	2013
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\| PubMed ID: 24280225	2014
The metazoan protein disaggregase and amyloid depolymerase system: Hsp110, Hsp70, Hsp40, and small heat shock proteins. Prion\| PubMed ID: 24401655	2013
Potentiated Hsp104 variants antagonize diverse proteotoxic misfolding events. Cell\| PubMed ID: 24439375	2014
ALS-associated mutation FUS-R521C causes DNA damage and RNA splicing defects. The Journal of clinical investigation\| PubMed ID: 24509083	2014
Reversing deleterious protein aggregation with re-engineered protein disaggregases. Cell cycle (Georgetown, Tex.)\| PubMed ID: 24694655	2014
The Surprising Role of Amyloid Fibrils in HIV Infection. Biology\| PubMed ID: 24832047	2012
A cellular system that degrades misfolded proteins and protects against neurodegeneration. Molecular cell\| PubMed ID: 24882209	2014
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.)\| PubMed ID: 25002999	2013
Potentiated Hsp104 variants suppress toxicity of diverse neurodegenerative disease-linked proteins. Disease models & mechanisms\| PubMed ID: 25062688	2014
Specific aromatic foldamers potently inhibit spontaneous and seeded Aβ42 and Aβ43 fibril assembly. The Biochemical journal\| PubMed ID: 25142005	2014
Suramin inhibits hsp104 ATPase and disaggregase activity. PloS one\| PubMed ID: 25299406	2014
Counteracting Semen-mediated Enhancement of HIV Infection and Enveloped Virus Infection by a Lysine-specific Molecular Tweezer. AIDS research and human retroviruses\| PubMed ID: 25357741	2014