James Shorter

James Shorter

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Spiraling in Control: Structures and Mechanisms of the Hsp104 Disaggregase.

Cold Spring Harb Perspect Biol 2019 Aug 1;11(8). Epub 2019 Aug 1.

Department of Biochemistry and Biophysics; and the Institute for Neurodegenerative Diseases, University of California San Francisco, San Francisco, California 94158.

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http://dx.doi.org/10.1101/cshperspect.a034033DOI Listing
August 2019

Engineered protein disaggregases mitigate toxicity of aberrant prion-like fusion proteins underlying sarcoma.

J Biol Chem 2019 Jul 5;294(29):11286-11296. Epub 2019 Jun 5.

Department of Chemistry, Washington University, St. Louis, Missouri 63130

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http://dx.doi.org/10.1074/jbc.RA119.009494DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6643043PMC
July 2019

Structural basis for substrate gripping and translocation by the ClpB AAA+ disaggregase.

Nat Commun 2019 06 3;10(1):2393. Epub 2019 Jun 3.

Department of Biochemistry and Biophysics, Institute for Neurodegenerative Diseases, University of California, San Francisco, CA, 94158, USA.

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http://dx.doi.org/10.1038/s41467-019-10150-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6546751PMC
June 2019

The molecular language of membraneless organelles.

J Biol Chem 2019 May 25;294(18):7115-7127. Epub 2018 Jul 25.

From the Department of Biochemistry and Biophysics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania 19104

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http://dx.doi.org/10.1074/jbc.TM118.001192DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6509512PMC
May 2019

Phase separation of RNA-binding proteins in physiology and disease: An introduction to the JBC Reviews thematic series.

Authors:
James Shorter

J Biol Chem 2019 May 4;294(18):7113-7114. Epub 2019 Apr 4.

From the Department of Biochemistry and Biophysics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania 19104

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http://www.jbc.org/lookup/doi/10.1074/jbc.REV119.007944
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http://dx.doi.org/10.1074/jbc.REV119.007944DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6509514PMC
May 2019

Hsp104 and Potentiated Variants Can Operate as Distinct Nonprocessive Translocases.

Biophys J 2019 May 5;116(10):1856-1872. Epub 2019 Apr 5.

University of Alabama at Birmingham, Chemistry Department, Birmingham, Alabama. Electronic address:

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http://dx.doi.org/10.1016/j.bpj.2019.03.035DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6531783PMC
May 2019

Therapeutic Dissolution of Aberrant Phases by Nuclear-Import Receptors.

Trends Cell Biol 2019 Apr 16;29(4):308-322. Epub 2019 Jan 16.

Department of Biochemistry and Biophysics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA; Biochemistry and Molecular Biophysics Graduate Group, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA. Electronic address:

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https://linkinghub.elsevier.com/retrieve/pii/S09628924183021
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http://dx.doi.org/10.1016/j.tcb.2018.12.004DOI Listing
April 2019

RNA Binding Antagonizes Neurotoxic Phase Transitions of TDP-43.

Neuron 2019 Apr 27;102(2):321-338.e8. Epub 2019 Feb 27.

Department of Neurobiology, University of Pittsburgh School of Medicine, Pittsburgh, PA 15213, USA; LiveLikeLou Center for ALS Research, University of Pittsburgh Brain Institute, Pittsburgh, PA 15213, USA; Center for Neuroscience, University of Pittsburgh, Pittsburgh, PA 15213, USA; Center for Protein Conformational Diseases, Kenneth P. Dietrich School of Arts and Sciences, University of Pittsburgh, Pittsburgh, PA 15213, USA. Electronic address:

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https://linkinghub.elsevier.com/retrieve/pii/S08966273193007
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http://dx.doi.org/10.1016/j.neuron.2019.01.048DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6472983PMC
April 2019

Cytoplasmic TDP-43 De-mixing Independent of Stress Granules Drives Inhibition of Nuclear Import, Loss of Nuclear TDP-43, and Cell Death.

Neuron 2019 Apr 7;102(2):339-357.e7. Epub 2019 Mar 7.

Ludwig Institute for Cancer Research, University of California at San Diego, La Jolla, CA 92093, USA; Department of Cellular and Molecular Medicine, University of California at San Diego, La Jolla, CA 92093, USA. Electronic address:

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https://linkinghub.elsevier.com/retrieve/pii/S08966273193017
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http://dx.doi.org/10.1016/j.neuron.2019.02.038DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6548321PMC
April 2019

Hydrogen exchange reveals Hsp104 architecture, structural dynamics, and energetics in physiological solution.

Proc Natl Acad Sci U S A 2019 04 27;116(15):7333-7342. Epub 2019 Mar 27.

Johnson Research Foundation, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104;

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http://www.pnas.org/lookup/doi/10.1073/pnas.1816184116
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http://dx.doi.org/10.1073/pnas.1816184116DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6462101PMC
April 2019

AAA+ Protein-Based Technologies to Counter Neurodegenerative Disease.

Biophys J 2019 Apr 22;116(8):1380-1385. Epub 2019 Mar 22.

Department of Biochemistry and Biophysics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania; Graduate Group in Biochemistry and Molecular Biophysics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania. Electronic address:

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https://linkinghub.elsevier.com/retrieve/pii/S00063495193019
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http://dx.doi.org/10.1016/j.bpj.2019.03.007DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6486517PMC
April 2019

Structure of Calcarisporiella thermophila Hsp104 Disaggregase that Antagonizes Diverse Proteotoxic Misfolding Events.

Structure 2019 Mar 27;27(3):449-463.e7. Epub 2018 Dec 27.

Midwest Center for Structural Genomics, Argonne National Laboratory, Argonne, IL 60439, USA; Structural Biology Center, Biosciences Division, Argonne National Laboratory, Argonne, IL 60439, USA; Center for Structural Genomics of Infectious Diseases, Consortium for Advanced Science and Engineering, University of Chicago, Chicago, IL 60637, USA; Department of Biochemistry and Molecular Biology, University of Chicago, Chicago, IL 60637, USA. Electronic address:

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http://dx.doi.org/10.1016/j.str.2018.11.001DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6403000PMC
March 2019

TDP-43 shapeshifts to encipher FTD severity.

Nat Neurosci 2019 01;22(1):3-5

Department of Biochemistry and Biophysics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA.

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http://www.nature.com/articles/s41593-018-0299-6
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http://dx.doi.org/10.1038/s41593-018-0299-6DOI Listing
January 2019

3' UTRs in the Eye of the TIGER.

Dev Cell 2018 12;47(5):544-546

Department of Biochemistry and Biophysics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA 19104, USA. Electronic address:

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http://dx.doi.org/10.1016/j.devcel.2018.11.022DOI Listing
December 2018

Poly(ADP-ribose) Engages the TDP-43 Nuclear-Localization Sequence to Regulate Granulo-Filamentous Aggregation.

Biochemistry 2018 12 17;57(51):6923-6926. Epub 2018 Dec 17.

Department of Biology , University of Pennsylvania , Philadelphia , Pennsylvania 19104 , United States.

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http://dx.doi.org/10.1021/acs.biochem.8b00910DOI Listing
December 2018

Editorial: The Role of AAA+ Proteins in Protein Repair and Degradation.

Front Mol Biosci 2018 2;5:85. Epub 2018 Oct 2.

Department of Biochemistry, University of Toronto, Toronto, ON, Canada.

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http://dx.doi.org/10.3389/fmolb.2018.00085DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6176779PMC
October 2018

Poly(ADP-Ribose) Prevents Pathological Phase Separation of TDP-43 by Promoting Liquid Demixing and Stress Granule Localization.

Mol Cell 2018 09 9;71(5):703-717.e9. Epub 2018 Aug 9.

Department of Biology, University of Pennsylvania, Philadelphia, PA 19104, USA. Electronic address:

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http://dx.doi.org/10.1016/j.molcel.2018.07.002DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6128762PMC
September 2018

Enhancement of Ebola virus infection by seminal amyloid fibrils.

Proc Natl Acad Sci U S A 2018 07 25;115(28):7410-7415. Epub 2018 Jun 25.

Department of Microbiology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104;

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http://dx.doi.org/10.1073/pnas.1721646115DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6048544PMC
July 2018

Molecular Dissection of FUS Points at Synergistic Effect of Low-Complexity Domains in Toxicity.

Cell Rep 2018 Jul;24(3):529-537.e4

Experimental Neurology, Department of Neurosciences, and Leuven Brain Institute (LBI), KU Leuven-University of Leuven, 3000 Leuven, Belgium; Laboratory of Neurobiology, Center for Brain & Disease Research, VIB, 3000 Leuven, Belgium. Electronic address:

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http://dx.doi.org/10.1016/j.celrep.2018.06.070DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6077250PMC
July 2018

Protein Phase Separation: A New Phase in Cell Biology.

Trends Cell Biol 2018 06 27;28(6):420-435. Epub 2018 Mar 27.

MTA-DE Laboratory of Protein Dynamics, Department of Biochemistry and Molecular Biology, University of Debrecen, Debrecen, Hungary. Electronic address:

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http://dx.doi.org/10.1016/j.tcb.2018.02.004DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6034118PMC
June 2018

Amyloid assembly and disassembly.

J Cell Sci 2018 Apr 13;131(8). Epub 2018 Apr 13.

Department of Biochemistry and Biophysics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA 19104, USA

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http://dx.doi.org/10.1242/jcs.189928DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5963839PMC
April 2018

FUS Regulates Activity of MicroRNA-Mediated Gene Silencing.

Mol Cell 2018 03;69(5):787-801.e8

Department of Biochemistry and Molecular Biology, Bloomberg School of Public Health, Johns Hopkins University, Baltimore, MD 21205, USA; Department of Neuroscience, School of Medicine, Johns Hopkins University, Baltimore, MD 21205, USA. Electronic address:

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http://dx.doi.org/10.1016/j.molcel.2018.02.001DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5836505PMC
March 2018

Ubiquilin 2: Shuttling Clients Out of Phase?

Mol Cell 2018 03;69(6):919-921

Department of Biochemistry and Biophysics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA 19104, USA. Electronic address:

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http://dx.doi.org/10.1016/j.molcel.2018.02.030DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5966015PMC
March 2018

Liquidizing FUS via prion-like domain phosphorylation.

Authors:
James Shorter

EMBO J 2017 10 28;36(20):2925-2927. Epub 2017 Sep 28.

Department of Biochemistry and Biophysics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA.

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http://dx.doi.org/10.15252/embj.201798078DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5641903PMC
October 2017

Biology and Pathobiology of TDP-43 and Emergent Therapeutic Strategies.

Cold Spring Harb Perspect Med 2017 Sep 1;7(9). Epub 2017 Sep 1.

Department of Biochemistry and Biophysics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania 19104.

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http://dx.doi.org/10.1101/cshperspect.a024554DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5580514PMC
September 2017

Prion-like Domains Program Ewing's Sarcoma.

Authors:
James Shorter

Cell 2017 09;171(1):30-31

Department of Biochemistry and Biophysics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA 19104, USA. Electronic address:

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http://dx.doi.org/10.1016/j.cell.2017.09.010DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5639709PMC
September 2017

Designer protein disaggregases to counter neurodegenerative disease.

Authors:
James Shorter

Curr Opin Genet Dev 2017 Jun 14;44:1-8. Epub 2017 Feb 14.

Department of Biochemistry and Biophysics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA 19104, United States of America. Electronic address:

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http://dx.doi.org/10.1016/j.gde.2017.01.008DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5447488PMC
June 2017

Neurodegenerative disease: models, mechanisms, and a new hope.

Dis Model Mech 2017 05;10(5):499-502

Department of Biochemistry & Biophysics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA 19104, USA

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http://dx.doi.org/10.1242/dmm.030205DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5451177PMC
May 2017

FUS inclusions disrupt RNA localization by sequestering kinesin-1 and inhibiting microtubule detyrosination.

J Cell Biol 2017 04 15;216(4):1015-1034. Epub 2017 Mar 15.

Laboratory of Cellular and Molecular Biology, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892

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http://dx.doi.org/10.1083/jcb.201608022DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5379945PMC
April 2017

Avidity for Polypeptide Binding by Nucleotide-Bound Hsp104 Structures.

Biochemistry 2017 04 10;56(15):2071-2075. Epub 2017 Apr 10.

Department of Chemistry, University of Alabama at Birmingham , Birmingham, Alabama 35294, United States.

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http://dx.doi.org/10.1021/acs.biochem.7b00225DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5649362PMC
April 2017

RNA-binding proteins with prion-like domains in health and disease.

Biochem J 2017 04 7;474(8):1417-1438. Epub 2017 Apr 7.

Department of Biochemistry and Biophysics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA 19104, U.S.A.

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http://dx.doi.org/10.1042/BCJ20160499DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5639257PMC
April 2017

Protein-Remodeling Factors As Potential Therapeutics for Neurodegenerative Disease.

Front Neurosci 2017 28;11:99. Epub 2017 Feb 28.

Department of Biochemistry and Biophysics, Perelman School of Medicine at the University of Pennsylvania Philadelphia, PA, USA.

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http://dx.doi.org/10.3389/fnins.2017.00099DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5328956PMC
February 2017

Susan Lee Lindquist (1949-2016).

Nature 2016 11;540(7631):40

Stanford University, Stanford, California, USA.

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http://dx.doi.org/10.1038/540040aDOI Listing
November 2016

Prion-like domains as epigenetic regulators, scaffolds for subcellular organization, and drivers of neurodegenerative disease.

Brain Res 2016 09 18;1647:9-18. Epub 2016 Mar 18.

Department of Biochemistry and Biophysics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA 19104, United States of America; Biochemistry and Molecular Biophysics Graduate Group, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA 19104, United States of America. Electronic address:

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http://dx.doi.org/10.1016/j.brainres.2016.02.037DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5003744PMC
September 2016

Spiral architecture of the Hsp104 disaggregase reveals the basis for polypeptide translocation.

Nat Struct Mol Biol 2016 09 1;23(9):830-7. Epub 2016 Aug 1.

Department of Biological Chemistry, Life Sciences Institute, University of Michigan, Ann Arbor, Michigan, USA.

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http://dx.doi.org/10.1038/nsmb.3277DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5509435PMC
September 2016

Mechanistic and Structural Insights into the Prion-Disaggregase Activity of Hsp104.

J Mol Biol 2016 05 1;428(9 Pt B):1870-85. Epub 2015 Dec 1.

Department of Biochemistry and Biophysics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA 19104, USA. Electronic address:

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http://dx.doi.org/10.1016/j.jmb.2015.11.016DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4860052PMC
May 2016

Membraneless organelles: Phasing in and out.

Authors:
James Shorter

Nat Chem 2016 05;8(6):528-30

Department of Biochemistry and Biophysics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania 19104, USA.

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http://dx.doi.org/10.1038/nchem.2534DOI Listing
May 2016

Engineering therapeutic protein disaggregases.

Authors:
James Shorter

Mol Biol Cell 2016 05;27(10):1556-60

Department of Biochemistry and Biophysics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA 19104

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http://dx.doi.org/10.1091/mbc.E15-10-0693DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4865313PMC
May 2016

Reversing deleterious protein aggregation with re-engineered protein disaggregases.

Cell Cycle 2014 2;13(9):1379-83. Epub 2014 Apr 2.

Department of Biochemistry and Biophysics; Perelman School of Medicine at the University of Pennsylvania; Philadelphia, PA USA.

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http://dx.doi.org/10.4161/cc.28709DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4050135PMC
April 2016

Mechanistic Insights into Hsp104 Potentiation.

J Biol Chem 2016 Mar 8;291(10):5101-15. Epub 2016 Jan 8.

From the Department of Biochemistry and Biophysics and the Pharmacology Graduate Group, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania 19104

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http://dx.doi.org/10.1074/jbc.M115.707976DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4777845PMC
March 2016

Engineering and Evolution of Molecular Chaperones and Protein Disaggregases with Enhanced Activity.

Front Mol Biosci 2016 15;3. Epub 2016 Mar 15.

Department of Biochemistry and Biophysics, Perelman School of Medicine at the University of PennsylvaniaPhiladelphia, PA, USA; Biochemistry and Molecular Biophysics Graduate Group, Perelman School of Medicine at the University of PennsylvaniaPhiladelphia, PA, USA.

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http://dx.doi.org/10.3389/fmolb.2016.00008DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4791398PMC
March 2016

Engineering enhanced protein disaggregases for neurodegenerative disease.

Prion 2015 ;9(2):90-109

a Department of Biochemistry and Biophysics ; Perelman School of Medicine at the University of Pennsylvania ; Philadelphia , PA USA.

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http://dx.doi.org/10.1080/19336896.2015.1020277DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4601286PMC
February 2016

Suramin inhibits Hsp104 ATPase and disaggregase activity.

PLoS One 2014 9;9(10):e110115. Epub 2014 Oct 9.

Department of Biochemistry and Biophysics, University of Pennsylvania, Philadelphia, Pennsylvania, United States of America; Pharmacology Graduate Group, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, United States of America.

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0110115PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4192545PMC
December 2015

Disparate Mutations Confer Therapeutic Gain of Hsp104 Function.

ACS Chem Biol 2015 Dec 15;10(12):2672-9. Epub 2015 Oct 15.

Department of Biochemistry and Biophysics, ‡Cell and Molecular Biology Graduate Group, Perelman School of Medicine at the University of Pennsylvania , Philadelphia, Pennsylvania 19104, United States.

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http://dx.doi.org/10.1021/acschembio.5b00765DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4684741PMC
December 2015

Fleeting Amyloid-like Forms of Rim4 Ensure Meiotic Fidelity.

Cell 2015 Oct;163(2):275-6

Department of Biochemistry and Biophysics, Perelman School of Medicine at The University of Pennsylvania, Philadelphia, PA 19104, USA; Neuroscience Graduate Group, Perelman School of Medicine at The University of Pennsylvania, Philadelphia, PA 19104, USA. Electronic address:

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http://dx.doi.org/10.1016/j.cell.2015.09.049DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4653133PMC
October 2015

It's Raining Liquids: RNA Tunes Viscoelasticity and Dynamics of Membraneless Organelles.

Mol Cell 2015 Oct;60(2):189-92

Department of Biochemistry and Biophysics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA 19104, USA. Electronic address:

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http://dx.doi.org/10.1016/j.molcel.2015.10.006DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4653132PMC
October 2015

Repurposing Hsp104 to Antagonize Seminal Amyloid and Counter HIV Infection.

Chem Biol 2015 Aug 6;22(8):1074-86. Epub 2015 Aug 6.

Department of Biochemistry and Biophysics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA; Pharmacology Graduate Group, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA; Cell and Molecular Biology Graduate Group, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA. Electronic address:

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http://dx.doi.org/10.1016/j.chembiol.2015.07.007DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4546571PMC
August 2015

Epigallocatechin-3-gallate rapidly remodels PAP85-120, SEM1(45-107), and SEM2(49-107) seminal amyloid fibrils.

Biol Open 2015 Aug 28;4(9):1206-12. Epub 2015 Aug 28.

Department of Biochemistry and Biophysics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA 19104, USA Pharmacology Graduate Group, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA 19104, USA

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http://dx.doi.org/10.1242/bio.010215DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4582112PMC
August 2015

The metazoan protein disaggregase and amyloid depolymerase system: Hsp110, Hsp70, Hsp40, and small heat shock proteins.

Prion 2013 Nov-Dec;7(6):457-63. Epub 2014 Jan 8.

Department of Biochemistry and Biophysics; 805b Stellar-Chance Laboratories; Perelman School of Medicine; University of Pennsylvania; Philadelphia, PA USA.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4201613PMC
http://dx.doi.org/10.4161/pri.27531DOI Listing
March 2015

The Hsp104 N-terminal domain enables disaggregase plasticity and potentiation.

Mol Cell 2015 Mar 22;57(5):836-849. Epub 2015 Jan 22.

Department of Biochemistry and Biophysics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA; Biochemistry and Molecular Biophysics Graduate Group, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA. Electronic address:

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http://dx.doi.org/10.1016/j.molcel.2014.12.021DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4623595PMC
March 2015

Specific aromatic foldamers potently inhibit spontaneous and seeded Aβ42 and Aβ43 fibril assembly.

Biochem J 2014 Nov;464(1):85-98

‡Department of Pharmaceutical Chemistry, University of California, San Francisco, CVRI-MC Box 3122, San Francisco, CA 94158-9001 U.S.A.

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http://biochemj.org/lookup/doi/10.1042/BJ20131609
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http://dx.doi.org/10.1042/BJ20131609DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4209162PMC
November 2014

Isolating potentiated Hsp104 variants using yeast proteinopathy models.

J Vis Exp 2014 Nov 11(93):e52089. Epub 2014 Nov 11.

Department of Biochemistry and Biophysics, Perelman School of Medicine at the University of Pennsylvania;

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http://dx.doi.org/10.3791/52089DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4238040PMC
November 2014

Potentiated Hsp104 variants suppress toxicity of diverse neurodegenerative disease-linked proteins.

Dis Model Mech 2014 Oct 25;7(10):1175-84. Epub 2014 Jul 25.

Department of Biochemistry and Biophysics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA 19104, USA.

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http://dx.doi.org/10.1242/dmm.016113DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4174528PMC
October 2014

Disease mutations in the prion-like domains of hnRNPA1 and hnRNPA2/B1 introduce potent steric zippers that drive excess RNP granule assembly.

Rare Dis 2013 29;1:e25200. Epub 2013 May 29.

Department of Developmental Neurobiology; St Jude Children's Research Hospital; Memphis, TN USA.

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http://dx.doi.org/10.4161/rdis.25200DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3932941PMC
July 2014

A cellular system that degrades misfolded proteins and protects against neurodegeneration.

Mol Cell 2014 Jul 29;55(1):15-30. Epub 2014 May 29.

Department of Cancer Biology and Abramson Family Cancer Research Institute, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA. Electronic address:

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http://dx.doi.org/10.1016/j.molcel.2014.04.030DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4445634PMC
July 2014

Hsp104 suppresses polyglutamine-induced degeneration post onset in a drosophila MJD/SCA3 model.

PLoS Genet 2013 5;9(9):e1003781. Epub 2013 Sep 5.

Department of Biology, University of Pennsylvania, Philadelphia, Pennsylvania, United States of America ; Department of Biochemistry and Biophysics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, United States of America ; Neuroscience Graduate Group, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, United States of America.

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http://dx.doi.org/10.1371/journal.pgen.1003781DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3764203PMC
March 2014

Potentiated Hsp104 variants antagonize diverse proteotoxic misfolding events.

Cell 2014 Jan;156(1-2):170-82

Department of Biochemistry and Biophysics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA 19104, USA; Biochemistry and Molecular Biophysics Graduate Group, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA 19104, USA; Pharmacology Graduate Group, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA 19104, USA. Electronic address:

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https://linkinghub.elsevier.com/retrieve/pii/S00928674130154
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http://dx.doi.org/10.1016/j.cell.2013.11.047DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3909490PMC
January 2014

Fission yeast does not age under favorable conditions, but does so after stress.

Curr Biol 2013 Oct 12;23(19):1844-52. Epub 2013 Sep 12.

Max Planck Institute of Molecular Cell Biology and Genetics, Pfotenhauerstrasse 108, 01307 Dresden, Germany.

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http://dx.doi.org/10.1016/j.cub.2013.07.084DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4620659PMC
October 2013

RNA-binding proteins with prion-like domains in ALS and FTLD-U.

Prion 2011 Jul-Sep;5(3):179-87. Epub 2011 Jul 1.

Department of Cell and Developmental Biology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, USA.

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http://dx.doi.org/10.4161/pri.5.3.17230DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3226045PMC
April 2013

Stress granules as crucibles of ALS pathogenesis.

J Cell Biol 2013 Apr;201(3):361-72

Medical Scientist Training Program and, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA 19104, USA.

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http://dx.doi.org/10.1083/jcb.201302044DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3639398PMC
April 2013

Operational plasticity enables hsp104 to disaggregate diverse amyloid and nonamyloid clients.

Cell 2012 Nov;151(4):778-793

Department of Biochemistry and Biophysics, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA; Biochemistry and Molecular Biophysics Graduate Group, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA; Neuroscience Graduate Group, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA. Electronic address:

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http://dx.doi.org/10.1016/j.cell.2012.09.038DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3496281PMC
November 2012

Hsp104 drives "protein-only" positive selection of Sup35 prion strains encoding strong [PSI(+)].

Chem Biol 2012 Nov;19(11):1400-10

Department of Biochemistry and Biophysics, 805b Stellar-Chance Laboratories, Perelman School of Medicine, University of Pennsylvania, 422 Curie Boulevard, Philadelphia, PA 19104, USA.

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http://dx.doi.org/10.1016/j.chembiol.2012.09.013DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3508465PMC
November 2012

Small heat shock proteins potentiate amyloid dissolution by protein disaggregases from yeast and humans.

PLoS Biol 2012 19;10(6):e1001346. Epub 2012 Jun 19.

Boston Biomedical Research Institute, Watertown, Massachusetts, United States of America.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3378601PMC
October 2012

RNA-Binding Proteins in Amyotrophic Lateral Sclerosis and Neurodegeneration.

Neurol Res Int 2012 19;2012:432780. Epub 2012 Apr 19.

Biochemistry and Molecular Biophysics Graduate Group, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA.

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http://dx.doi.org/10.1155/2012/432780DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3423945PMC
August 2012

The tip of the iceberg: RNA-binding proteins with prion-like domains in neurodegenerative disease.

Brain Res 2012 Jun 21;1462:61-80. Epub 2012 Jan 21.

Boston Biomedical Research Institute, 64 Grove St., Watertown, MA 02472, USA.

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http://dx.doi.org/10.1016/j.brainres.2012.01.016DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3372647PMC
June 2012

The Surprising Role of Amyloid Fibrils in HIV Infection.

Biology (Basel) 2012 May 29;1(1):58-80. Epub 2012 May 29.

Pharmacology Graduate Group, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA 19104, USA.

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http://dx.doi.org/10.3390/biology1010058DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4011035PMC
May 2012

The mammalian disaggregase machinery: Hsp110 synergizes with Hsp70 and Hsp40 to catalyze protein disaggregation and reactivation in a cell-free system.

Authors:
James Shorter

PLoS One 2011 14;6(10):e26319. Epub 2011 Oct 14.

Stellar-Chance Laboratories, Department of Biochemistry and Biophysics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, United States of America.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3194798PMC
February 2012

The elusive middle domain of Hsp104 and ClpB: location and function.

Biochim Biophys Acta 2012 Jan 24;1823(1):29-39. Epub 2011 Jul 24.

Department of Biochemistry and Biophysics, Perelman School of Medicine at The University of Pennsylvania, 805b Stellar-Chance Laboratories, 422 Curie Boulevard, Philadelphia, PA 19104, USA.

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http://dx.doi.org/10.1016/j.bbamcr.2011.07.014DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3219823PMC
January 2012

Purification of hsp104, a protein disaggregase.

J Vis Exp 2011 Sep 30(55). Epub 2011 Sep 30.

Department of Biochemistry and Biophysics, University of Pennsylvania, USA.

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http://dx.doi.org/10.3791/3190DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3230206PMC
September 2011

Countering amyloid polymorphism and drug resistance with minimal drug cocktails.

Prion 2010 Oct-Dec;4(4):244-51. Epub 2010 Oct 12.

Boston Biomedical Research Institute, Watertown, MA, USA.

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http://dx.doi.org/10.4161/pri.4.4.13597DOI Listing
June 2011

Molecular determinants and genetic modifiers of aggregation and toxicity for the ALS disease protein FUS/TLS.

PLoS Biol 2011 Apr 26;9(4):e1000614. Epub 2011 Apr 26.

Department of Cell and Developmental Biology, The University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania, United States of America.

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http://dx.doi.org/10.1371/journal.pbio.1000614DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3082519PMC
April 2011

Comparison of two village primary schools in northern Tanzania affected by fluorosis.

Int Health 2010 Dec;2(4):269-74

Department of Civil Engineering, Newcastle University, Newcastle upon Tyne, UK.

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http://dx.doi.org/10.1016/j.inhe.2010.09.010DOI Listing
December 2010

Emergence and natural selection of drug-resistant prions.

Authors:
James Shorter

Mol Biosyst 2010 Jul 27;6(7):1115-30. Epub 2010 Apr 27.

Department of Biochemistry and Biophysics, University of Pennsylvania School of Medicine, 805b Stellar-Chance Laboratories, 422 Curie Boulevard, Philadelphia, PA 19104, USA.

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http://dx.doi.org/10.1039/c004550kDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2936920PMC
July 2010

Prion-like disorders: blurring the divide between transmissibility and infectivity.

J Cell Sci 2010 Apr;123(Pt 8):1191-201

Department of Biochemistry and Biophysics, University of Pennsylvania School of Medicine, 805b Stellar-Chance Laboratories, 422 Curie Boulevard, Philadelphia, PA 19104, USA.

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http://dx.doi.org/10.1242/jcs.051672DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2848109PMC
April 2010

Applying Hsp104 to protein-misfolding disorders.

Biochem Cell Biol 2010 Feb;88(1):1-13

Department of Biochemistry and Biophysics, University of Pennsylvania School of Medicine, 805b Stellar-Chance Laboratories, 422 Curie Boulevard, Philadelphia, PA 19104, USA.

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http://dx.doi.org/10.1139/o09-121DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2933416PMC
February 2010

N-terminal domains elicit formation of functional Pmel17 amyloid fibrils.

J Biol Chem 2009 Dec;284(51):35543-55

Departments of Pathology and Laboratory Medicine, University of Pennsylvania, Philadelphia, Pennsylvania 19104, USA.

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http://dx.doi.org/10.1074/jbc.M109.047449DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2790984PMC
December 2009

A synergistic small-molecule combination directly eradicates diverse prion strain structures.

Nat Chem Biol 2009 Dec 1;5(12):936-46. Epub 2009 Nov 1.

Department of Biochemistry and Biophysics, University of Pennsylvania School of Medicine, Philadelphia, USA.

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http://dx.doi.org/10.1038/nchembio.246DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2909773PMC
December 2009

TDP-43 is intrinsically aggregation-prone, and amyotrophic lateral sclerosis-linked mutations accelerate aggregation and increase toxicity.

J Biol Chem 2009 Jul 22;284(30):20329-39. Epub 2009 May 22.

Department of Cell and Developmental Biology, the University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA.

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http://dx.doi.org/10.1074/jbc.M109.010264DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2740458PMC
July 2009

Prion proteostasis: Hsp104 meets its supporting cast.

Prion 2008 Oct-Dec;2(4):135-40. Epub 2008 Oct 22.

Department of Biochemistry and Biophysics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA.

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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2658762PMC
http://dx.doi.org/10.4161/pri.2.4.7952DOI Listing
March 2009

The Mad2 partial unfolding model: regulating mitosis through Mad2 conformational switching.

J Cell Biol 2008 Dec 24;183(5):761-8. Epub 2008 Nov 24.

Department of Biochemistry and Biophysics, University of Pennsylvania, Philadelphia, PA 19104, USA.

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http://dx.doi.org/10.1083/jcb.200808122DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2592820PMC
December 2008

A PDZ-binding motif controls basolateral targeting of syndecan-1 along the biosynthetic pathway in polarized epithelial cells.

Traffic 2008 Nov 30;9(11):1915-24. Epub 2008 Jul 30.

Department of Cell Biology, Ludwig Institute for Cancer Research, Yale University School of Medicine, New Haven, CT 06520, USA.

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http://dx.doi.org/10.1111/j.1600-0854.2008.00805.xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2820280PMC
November 2008

Hsp104, Hsp70 and Hsp40 interplay regulates formation, growth and elimination of Sup35 prions.

EMBO J 2008 Oct 2;27(20):2712-24. Epub 2008 Oct 2.

Department of Biochemistry and Biophysics, University of Pennsylvania School of Medicine, Stellar-Chance Laboratories, Philadelphia, PA 19104, USA.

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http://dx.doi.org/10.1038/emboj.2008.194DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2572177PMC
October 2008

Hsp104 antagonizes alpha-synuclein aggregation and reduces dopaminergic degeneration in a rat model of Parkinson disease.

J Clin Invest 2008 Sep;118(9):3087-97

Wallenberg Neuroscience Center, Division of Neurobiology, Department of Experimental Medical Science, Lund University, Lund, Sweden.

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http://www.jci.org/articles/view/35781
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2515383PMC
September 2008

Escaping amyloid fate.

Nat Struct Mol Biol 2008 Jun;15(6):544-6

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June 2008