List of works - James Shorter

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

scientific article

Stress granules as crucibles of ALS pathogenesis.

scientific article published on April 2013

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

scientific article

Prions as adaptive conduits of memory and inheritance

scientific article

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

scientific article

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

scientific article

A yeast functional screen predicts new candidate ALS disease genes

scientific article published on 7 November 2011

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

scientific article published on April 15, 2010

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

scientific article

Protein Phase Separation: A New Phase in Cell Biology

scientific article published on 27 March 2018

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

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

scientific article

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

scientific article

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

scientific article published on 10 February 2014

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

scientific article published on September 2008

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

scientific article

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

scientific article published on June 19, 2012

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

scientific article

Potentiated Hsp104 variants antagonize diverse proteotoxic misfolding events

scientific article published on January 2014

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

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

scientific article published on November 2012

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

scientific article published on July 1, 2011

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

scientific article published on 15 June 2017

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

scientific article published on 7 April 2017

Hsp104: a weapon to combat diverse neurodegenerative disorders.

scientific article published on 05 December 2007

A cellular system that degrades misfolded proteins and protects against neurodegeneration

scientific article published on 29 May 2014

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

scientific article

N-terminal domains elicit formation of functional Pmel17 amyloid fibrils

scientific article published on December 2009

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

scientific article published on 01 April 2018

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

scientific article published on January 2000

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

scientific article

Applying Hsp104 to protein-misfolding disorders.

scientific article published on February 2010

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

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

scientific article published on May 2017

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

scientific article published on October 2015

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

scientific article

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

scientific article published on 24 July 2011

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

scientific article

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

scientific article published on November 2013

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

scientific article

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

scientific article

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

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

The molecular language of membraneless organelles

scientific article published on 25 July 2018

The Hsp104 N-terminal domain enables disaggregase plasticity and potentiation

scientific article

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

scholarly article

Chaperones in Neurodegeneration

scientific article

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

scientific article published in Nature

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

List of works by James Shorter

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

Stress granules as crucibles of ALS pathogenesis.

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

Prions as adaptive conduits of memory and inheritance

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

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

A yeast functional screen predicts new candidate ALS disease genes

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

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

Protein Phase Separation: A New Phase in Cell Biology

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

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

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

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

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

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

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

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

Potentiated Hsp104 variants antagonize diverse proteotoxic misfolding events

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

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

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

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

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

Hsp104: a weapon to combat diverse neurodegenerative disorders.

A cellular system that degrades misfolded proteins and protects against neurodegeneration

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

N-terminal domains elicit formation of functional Pmel17 amyloid fibrils

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

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

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

Applying Hsp104 to protein-misfolding disorders.

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

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

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

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

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

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

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

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

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

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

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

The molecular language of membraneless organelles

The Hsp104 N-terminal domain enables disaggregase plasticity and potentiation

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

Chaperones in Neurodegeneration

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

Prime Time for -Synuclein

Prion proteostasis: Hsp104 meets its supporting cast