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Author Details

James Shorter
Perelman School of Medicine at the University of Pennsylvania
1997
182
66
Nevan J Krogan (CM4AI)
PMIDPaper TitleJournal TitlePublished Year
36529289A minimal construct of nuclear-import receptor Karyopherin-β2 defines the regions critical for chaperone and disaggregation activity.J Biol Chem2023
37491983Introduction: Phase Separation.Chem Rev2023
37625404Tuning Hsp104 specificity to selectively detoxify α-synuclein.Mol Cell2023
37481159Cryo-EM Structure of the Full-length hnRNPA1 Amyloid Fibril.J Mol Biol2023
37732211Defining RNA oligonucleotides that reverse deleterious phase transitions of RNA-binding proteins with prion-like domains.bioRxiv2023
37115110Regulation of Biomolecular Condensates by Poly(ADP-ribose).Chem Rev2023
37438085Transcriptome-wide RNA binding analysis of C9orf72 poly(PR) dipeptides.Life Sci Alliance2023
37000196C-terminal frameshift variant of TDP-43 with pronounced aggregation-propensity causes rimmed vacuole myopathy but not ALS/FTD.Acta Neuropathol2023
36690068Phase Separation in Biology and Disease; Current Perspectives and Open Questions.J Mol Biol2023
36935218Repetitive elements in aging and neurodegeneration.Trends Genet2023
34115842Heterozygous variants of CLPB are a cause of severe congenital neutropenia.Blood2022
35549000Sequestration of TDP-43<sup>216-414</sup> Aggregates by Cytoplasmic Expression of the proSAAS Chaperone.ACS Chem Neurosci2022
35581326Heat shock protein Grp78/BiP/HspA5 binds directly to TDP-43 and mitigates toxicity associated with disease pathology.Sci Rep2022
35427496Flying under the radar: TMEM106B(120-254) fibrils break out in diverse neurodegenerative disorders.Cell2022
35484142Heterozygous frameshift variants in HNRNPA2B1 cause early-onset oculopharyngeal muscular dystrophy.Nat Commun2022
35356966AAA+ proteins: one motor, multiple ways to work.Biochem Soc Trans2022
35588748Sexually dimorphic RNA helicases DDX3X and DDX3Y differentially regulate RNA metabolism through phase separation.Mol Cell2022
36482422Nuclear import receptors are recruited by FG-nucleoporins to rescue hallmarks of TDP-43 proteinopathy.Mol Neurodegener2022
36186568Advanced Molecular Tweezers with Lipid Anchors against SARS-CoV-2 and Other Respiratory Viruses.JACS Au2022
35182479Poly(ADP-ribose) drives condensation of FUS via a transient interaction.Mol Cell2022
34880481Aggregates of TDP-43 protein spiral into view.Nature2022
35197626TDP-43 represses cryptic exon inclusion in the FTD-ALS gene UNC13A.Nature2022
34464655Nuclear-Import Receptors Counter Deleterious Phase Transitions in Neurodegenerative Disease.J Mol Biol2022
33387533Biochemical Timekeeping Via Reentrant Phase Transitions.J Mol Biol2021
33792567ALS-associated mutation FUS-R521C causes DNA damage and RNA splicing defects.J Clin Invest2021
33551183Open Access: A Role for p53 in c9ALS/FTD?Trends Genet2021
33549703Combating deleterious phase transitions in neurodegenerative disease.Biochim Biophys Acta Mol Cell Res2021
34408321DAXX represents a new type of protein-folding enabler.Nature2021
34380047TDP-43 condensation properties specify its RNA-binding and regulatory repertoire.Cell2021
34732320N-alpha-acetylation of Huntingtin protein increases its propensity to aggregate.J Biol Chem2021
33942880(Dis)Solving the problem of aberrant protein states.Dis Model Mech2021
34129784Higher-order organization of biomolecular condensates.Open Biol2021
34015263Tau heckles speckles: A pathogenic mechanism in tauopathy?Neuron2021
34291734Characterization of HNRNPA1 mutations defines diversity in pathogenic mechanisms and clinical presentation.JCI Insight2021
33446423FUS and TDP-43 Phases in Health and Disease.Trends Biochem Sci2021
31630970Loss of Dynamic RNA Interaction and Aberrant Phase Separation Induced by Two Distinct Types of ALS/FTD-Linked FUS Mutations.Mol Cell2020
33659495Expression and Purification of Recombinant Skd3 (Human ClpB) Protein and Tobacco Etch Virus (TEV) Protease from <i>Escherichia coli</i>.Bio Protoc2020
33659387Karyopherin-β2 Inhibits and Reverses Aggregation and Liquid-liquid Phase Separation of the ALS/FTD-Associated Protein FUS.Bio Protoc2020
31926785Emerging small-molecule therapeutic approaches for amyotrophic lateral sclerosis and frontotemporal dementia.Bioorg Med Chem Lett2020
32043626The clinical trial landscape in amyotrophic lateral sclerosis-Past, present, and future.Med Res Rev2020
31825716The extent of Ssa1/Ssa2 Hsp70 chaperone involvement in nuclear protein quality control degradation varies with the substrate.Mol Biol Cell2020
31882541Structural and mechanistic insights into Hsp104 function revealed by synchrotron X-ray footprinting.J Biol Chem2020
31734037Switching Condensates: The CTD Code Goes Liquid.Trends Biochem Sci2020
33338404ALS/FTLD-Linked Mutations in FUS Glycine Residues Cause Accelerated Gelation and Reduced Interactions with Wild-Type FUS.Mol Cell2020
33357437Nuclear Import Receptors Directly Bind to Arginine-Rich Dipeptide Repeat Proteins and Suppress Their Pathological Interactions.Cell Rep2020
33264628TRIM11 Prevents and Reverses Protein Aggregation and Rescues a Mouse Model of Parkinson's Disease.Cell Rep2020
32639008Arginine-rich dipeptide-repeat proteins as phase disruptors in C9-ALS/FTD.Emerg Top Life Sci2020
32573439Skd3 (human ClpB) is a potent mitochondrial protein disaggregase that is inactivated by 3-methylglutaconic aciduria-linked mutations.Elife2020
32878979<i>C9orf72</i> poly(GR) aggregation induces TDP-43 proteinopathy.Sci Transl Med2020
32792144Atomic Structures of Amyloid-β Oligomers Illuminate a Neurotoxic Mechanism.Trends Neurosci2020
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Collaborators

Aaron D Gitler
Stanford University School of Medicine
Co-authored papers 18
Susan Lindquist
Whitehead Institute for Biomedical Research
Co-authored papers 12
Zamia Diaz
Perelman School of Medicine, University of Pennsylvania
Co-authored papers 7
Daniel R Southworth
The Institute for Neurodegenerative Diseases, University of California San Francisco
Co-authored papers 6
Sami J Barmada
University of Michigan ann arbor
Co-authored papers 6
Dennis W Dickson
Mayo Clinic Jacksonville
Co-authored papers 5
Leonard Petrucelli
Co-authored papers 4
John Q Trojanowski
University of Pennsylvania
Co-authored papers 4
Rosa Rademakers
University of Antwerp
Co-authored papers 3
Nicolas L Fawzi
Brown University
Co-authored papers 3
Steven Finkbeiner
J. David Gladstone Institutes
Co-authored papers 3
Alexandrea N Rizo
University of California san francisco
Co-authored papers 3
Drew Weissman
Co-authored papers 3
Virginia M-Y Lee
Perelman School of Medicine at the University of Pennsylvania
Co-authored papers 3
Michael C Oldham
University of California san francisco
Co-authored papers 2
Helen R Saibil
Institute of Structural and Molecular Biology, University of London
Co-authored papers 2
Ronen Marmorstein
Perelman School of Medicine, University of Pennsylvania
Co-authored papers 2
Xiaodong Fang
University of Pennsylvania School of Medicine
Co-authored papers 2
Alessandra Chesi
Center for Spatial and Functional Genomics, The Children's Hospital of Philadelphia
Co-authored papers 2
Eric J Huang
University of California san francisco
Co-authored papers 2
Li-Huei Tsai
The Picower Institute for Learning and Memory, Massachusetts Institute of Technology
Co-authored papers 2
Amy A Tang
University of California san francisco
Co-authored papers 2
Tania F Gendron
Co-authored papers 2
Neill R Graff-Radford
Mayo Clinic Florida
Co-authored papers 2
Daniel C Link
Washington University School of Medicine
Co-authored papers 2
Anthony J Filiano
Co-authored papers 2
Eric Tse
School of Clinical Medicine, University of Hong Kong
Co-authored papers 2
Emily A Scarborough
Pennsylvania Muscle Institute, University of Pennsylvania Perelman School of Medicine
Co-authored papers 2
Eric D Nguyen
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Release 1.2