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

by James Shorter
Published 2011-01-01

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

by Ryan R Cupo, James Shorter
Published 2020-06-01

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

by Meredith E. Jackrel, James Shorter
Published 2014-10-01

The Surprising Role of Amyloid Fibrils in HIV Infection

by James Shorter, Laura M. Castellano
Published 2012-05-01

RNA-Binding Proteins in Amyotrophic Lateral Sclerosis and Neurodegeneration

by Scott E. Ugras, James Shorter
Published 2012-01-01

(Dis)Solving the problem of aberrant protein states

by Charlotte M. Fare, James Shorter
Published 2021-05-01

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

by Martin L Duennwald, Analisa Echeverria, James Shorter
Published 2012-01-01

Neurodegenerative disease: models, mechanisms, and a new hope

by Aaron D. Gitler, Paraminder Dhillon, James Shorter
Published 2017-05-01

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

by James Shorter, Walid A. Houry, Walid A. Houry
Published 2018-10-01

Suramin inhibits Hsp104 ATPase and disaggregase activity.

by Mariana P Torrente, Laura M Castellano, James Shorter
Published 2014-01-01

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

by Mimi Cushman-Nick, Nancy M Bonini, James Shorter
Published 2013-01-01

Higher-order organization of biomolecular condensates

by Charlotte M. Fare, Alexis Villani, Lauren E. Drake, James Shorter
Published 2021-06-01

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

by Laura M. Castellano, Rebecca M. Hammond, Veronica M. Holmes, Drew Weissman, James Shorter
Published 2015-09-01

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

by Heather Sadlish, Heike Rampelt, James Shorter, Renee D Wegrzyn, Claes Andréasson, Susan Lindquist, Bernd Bukau
Published 2008-01-01

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

by Zhihui Sun, Zamia Diaz, Xiaodong Fang, Michael P Hart, Alessandra Chesi, James Shorter, Aaron D Gitler
Published 2011-04-01

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

by Alexandrea N. Rizo, JiaBei Lin, Stephanie N. Gates, Eric Tse, Stephen M. Bart, Laura M. Castellano, Frank DiMaio, James Shorter, Daniel R. Southworth
Published 2019-06-01

TRIM11 Prevents and Reverses Protein Aggregation and Rescues a Mouse Model of Parkinson’s Disease

by Guixin Zhu, Dilshan S. Harischandra, Shivani Ghaisas, Pengfei Zhang, Wil Prall, Liangqian Huang, Chantal Maghames, Lili Guo, Esteban Luna, Korrie L. Mack, Mariana P. Torrente, Kelvin C. Luk, James Shorter, Xiaolu Yang
Published 2020-12-01

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

by Elke Bogaert, Steven Boeynaems, Masato Kato, Lin Guo, Thomas R. Caulfield, Jolien Steyaert, Wendy Scheveneels, Nathalie Wilmans, Wanda Haeck, Nicole Hersmus, Joost Schymkowitz, Frederic Rousseau, James Shorter, Patrick Callaerts, Wim Robberecht, Philip Van Damme, Ludo Van Den Bosch
Published 2018-07-01

Therapeutic genetic variation revealed in diverse Hsp104 homologs

by Zachary M March, Katelyn Sweeney, Hanna Kim, Xiaohui Yan, Laura M Castellano, Meredith E Jackrel, JiaBei Lin, Edward Chuang, Edward Gomes, Corey W Willicott, Karolina Michalska, Robert P Jedrzejczak, Andrzej Joachimiak, Kim A Caldwell, Guy A Caldwell, Ophir Shalem, James Shorter
Published 2020-12-01

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

by Amber Tariq, JiaBei Lin, Meredith E. Jackrel, Christina D. Hesketh, Peter J. Carman, Korrie L. Mack, Rachel Weitzman, Craig Gambogi, Oscar A. Hernandez Murillo, Elizabeth A. Sweeny, Esin Gurpinar, Adam L. Yokom, Stephanie N. Gates, Keolamau Yee, Saurabh Sudesh, Jacob Stillman, Alexandra N. Rizo, Daniel R. Southworth, James Shorter
Published 2019-08-01