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List of works by James Shorter

3' UTRs in the Eye of the TIGER

scientific article published on 01 December 2018

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

scientific article

A cellular system that degrades misfolded proteins and protects against neurodegeneration

scientific article published on 29 May 2014

A direct role for GRASP65 as a mitotically regulated Golgi stacking factor

scientific article

A molecular tweezer antagonizes seminal amyloids and HIV infection.

scientific article

A role for the vesicle tethering protein, p115, in the post-mitotic stacking of reassembling Golgi cisternae in a cell-free system.

scientific article published on July 1999

A yeast functional screen predicts new candidate ALS disease genes

scientific article published on 7 November 2011

AAA+ Protein-Based Technologies to Counter Neurodegenerative Disease

scientific article published on 22 March 2019

ALS-associated mutation FUS-R521C causes DNA damage and RNA splicing defects

scientific article published on 10 February 2014

Amyloid assembly and disassembly

scientific article published on 13 April 2018

Applying Hsp104 to protein-misfolding disorders.

scientific article published on February 2010

Avidity for Polypeptide Binding by Nucleotide-Bound Hsp104 Structures.

scientific article published on 5 April 2017

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

scientific article published on 5 December 2016

C9orf72 poly(GR) aggregation induces TDP-43 proteinopathy

scientific article published on 01 September 2020

CRISPR-Cas9 Screens Identify the RNA Helicase DDX3X as a Repressor of C9ORF72 (GGGGCC)n Repeat-Associated Non-AUG Translation

scientific article published on 03 October 2019

Chaperones in Neurodegeneration

scientific article

Conserved distal loop residues in the Hsp104 and ClpB middle domain contact nucleotide-binding domain 2 and enable Hsp70-dependent protein disaggregation.

scientific article

Countering amyloid polymorphism and drug resistance with minimal drug cocktails ⬇️

scientific article published on October 12, 2010

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

scientific article published on 07 March 2019

Designer protein disaggregases to counter neurodegenerative disease

scientific article published on 13 February 2017

Direct and selective elimination of specific prions and amyloids by 4,5-dianilinophthalimide and analogs

scholarly article

Discovery and characterization of an endogenous CXCR4 antagonist

scientific article

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

scientific article published on 29 May 2013

Disparate Mutations Confer Therapeutic Gain of Hsp104 Function.

scientific article published on 5 October 2015

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

scientific article published on 02 October 2018

Emergence and natural selection of drug-resistant prions

scientific article published on 27 April 2010

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

scientific article published on 05 June 2019

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

scientific article

Engineering enhanced protein disaggregases for neurodegenerative disease

scientific article

Engineering therapeutic protein disaggregases.

scientific article published on May 2016

Enhancement of Ebola virus infection by seminal amyloid fibrils

article published in the Proceedings of the National Academy of Sciences of the United States of America

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

scientific article

Escaping amyloid fate.

scientific article published in June 2008

FUS Regulates Activity of MicroRNA-Mediated Gene Silencing.

scientific article published in March 2018

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

scientific article published on 15 March 2017

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

scientific article

Fleeting Amyloid-like Forms of Rim4 Ensure Meiotic Fidelity

scientific article published on October 2015

GRASP55, a second mammalian GRASP protein involved in the stacking of Golgi cisternae in a cell-free system.

scientific article

Heterochromatin anomalies and double-stranded RNA accumulation underlie poly(PR) toxicity

article

Hsp104 and Potentiated Variants Can Operate as Distinct Nonprocessive Translocases

scientific article published on 05 April 2019

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

scientific article published on September 2008

Hsp104 catalyzes formation and elimination of self-replicating Sup35 prion conformers

scientific article

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

scientific article

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

scientific article

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

scientific article

Hsp104: a weapon to combat diverse neurodegenerative disorders.

scientific article published on 05 December 2007

Hsp110 chaperones regulate prion formation and propagation in S. cerevisiae by two discrete activities

scientific article

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

scientific article published on 27 March 2019

Inhibition of RNA lariat debranching enzyme suppresses TDP-43 toxicity in ALS disease models

scientific article

Isolating potentiated Hsp104 variants using yeast proteinopathy models

scientific article

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

scientific article published on October 2015

Liquidizing FUS via prion-like domain phosphorylation

scientific article published on 28 September 2017

Loss of Dynamic RNA Interaction and Aberrant Phase Separation Induced by Two Distinct Types of ALS/FTD-Linked FUS Mutations

scientific article published on 17 October 2019

Mechanistic Insights into Hsp104 Potentiation

scientific article published on 8 January 2016

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

scientific article

Membrane traffic: do cones mark sites of fission?

scientific article

Mining Disaggregase Sequence Space to Safely Counter TDP-43, FUS, and α-Synuclein Proteotoxicity

scientific article published on 01 August 2019

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

article

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

scientific article

Mutations in prion-like domains in hnRNPA2B1 and hnRNPA1 cause multisystem proteinopathy and ALS

scientific article

N-terminal domains elicit formation of functional Pmel17 amyloid fibrils

scientific article published on December 2009

Navigating the ClpB channel to solution.

scientific article

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

scientific article published on May 2017

Nuclear poly(ADP-ribose) activity is a therapeutic target in amyotrophic lateral sclerosis

scientific article published on 29 August 2018

Nuclear-Import Receptors Reverse Aberrant Phase Transitions of RNA-Binding Proteins with Prion-like Domains

scientific article published on 01 April 2018

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

scientific article published on November 2012

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

scientific article published on 04 April 2019

Phasing in and out

scientific article published on 01 May 2016

Phosphorylation of the vesicle-tethering protein p115 by a casein kinase II-like enzyme is required for Golgi reassembly from isolated mitotic fragments

scientific article

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

scientific article published on 09 August 2018

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

scientific article published on 17 December 2018

Potentiated Hsp104 variants antagonize diverse proteotoxic misfolding events

scientific article published on January 2014

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

scientific article

Potentiating Hsp104 activity via phosphomimetic mutations in the middle domain

scientific article published on 01 August 2018

Prime Time for -Synuclein

scientific article published on 01 March 2007

Prion proteostasis: Hsp104 meets its supporting cast

scientific article published on 22 October 2008

Prion-like Domains Program Ewing's Sarcoma

scientific article

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

scientific article

Prions as adaptive conduits of memory and inheritance

scientific article

Protein Phase Separation: A New Phase in Cell Biology

scientific article published on 27 March 2018

Protein-Remodeling Factors As Potential Therapeutics for Neurodegenerative Disease.

scientific article published on 28 February 2017

Purification of Hsp104, a Protein Disaggregase ⬇️

scientific article published on September 30, 2011

RNA Binding Antagonizes Neurotoxic Phase Transitions of TDP-43

scientific article published on 27 February 2019

RNA-Binding Proteins in Amyotrophic Lateral Sclerosis and Neurodegeneration.

scientific article published on 19 April 2012

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

scientific article published on July 1, 2011

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

scientific article published on 7 April 2017

Ratchet-like polypeptide translocation mechanism of the AAA+ disaggregase Hsp104.

scientific article published on 15 June 2017

Repurposing Hsp104 to Antagonize Seminal Amyloid and Counter HIV Infection.

scientific article published on 3 August 2015

Reversing deleterious protein aggregation with re-engineered protein disaggregases.

scientific article published on 2 April 2014

Sequential tethering of Golgins and catalysis of SNAREpin assembly by the vesicle-tethering protein p115

scientific article

Shock and awe: unleashing the heat shock response to treat Huntington disease.

scientific article published on 25 July 2011

Skd3 (human CLPB) is a potent mitochondrial protein disaggregase that is inactivated by 3-methylglutaconic aciduria-linked mutations

scientific article published on 23 June 2020

Small Heat Shock Proteins Potentiate Amyloid Dissolution by Protein Disaggregases from Yeast and Humans ⬇️

scientific article published on June 19, 2012

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

scientific article

Spiraling in Control: Structures and Mechanisms of the Hsp104 Disaggregase

scientific article published on 01 August 2019

Stress granules as crucibles of ALS pathogenesis.

scientific article published on April 2013

Structural and kinetic basis for the regulation and potentiation of Hsp104 function

scientific article published on 10 April 2020

Structural and mechanistic insights into Hsp104 function revealed by synchrotron X-ray footprinting

scientific article published on 27 December 2019

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

scientific article published on 3 June 2019

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

scientific article published on 27 December 2018

Suramin inhibits Hsp104 ATPase and disaggregase activity

scientific article

Susan Lee Lindquist (1949-2016).

scientific article published in November 2016

TDP-43 and RNA form amyloid-like myo-granules in regenerating muscle

scientific article published in Nature

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

scientific article

TDP-43 shapeshifts to encipher FTD severity

scientific article published on 01 January 2019

The Hsp104 N-terminal domain enables disaggregase plasticity and potentiation

scientific article

The Mammalian Disaggregase Machinery: Hsp110 Synergizes with Hsp70 and Hsp40 to Catalyze Protein Disaggregation and Reactivation in a Cell-Free System ⬇️

scientific article published on October 14, 2011

The Surprising Role of Amyloid Fibrils in HIV Infection

scientific article

The amino-terminal domain of the golgi protein giantin interacts directly with the vesicle-tethering protein p115.

scientific article published on January 2000

The clinical trial landscape in amyotrophic lateral sclerosis-Past, present, and future

scientific article published on 11 February 2020

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

scientific article published on 24 July 2011

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

scientific article published on November 2013

The molecular language of membraneless organelles.

scientific article published on 25 July 2018

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

scientific article

Therapeutic Dissolution of Aberrant Phases by Nuclear-Import Receptors

scientific article published on 16 January 2019

Therapeutic genetic variation revealed in diverse Hsp104 homologs

scientific article published on 15 December 2020

Ubiquilin 2: Shuttling Clients Out of Phase?

scholarly article by Ipsita Subudhi published in March 2018

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