Advanced search
Publication Cover
Prion Volume 9, 2015 - Issue 2
Submit an article Journal homepage
3,184
Views
63
CrossRef citations to date
0
Altmetric
Extra Views

Engineering enhanced protein disaggregases for neurodegenerative disease

Meredith E JackrelDepartment of Biochemistry and Biophysics; Perelman School of Medicine at the University of Pennsylvania; Philadelphia, PAUSA
&
James ShorterDepartment of Biochemistry and Biophysics; Perelman School of Medicine at the University of Pennsylvania; Philadelphia, PAUSACorrespondence[email protected]
Pages 90-109 | Received 21 Jan 2015, Accepted 12 Feb 2015, Published online: 21 May 2015

Citations (63)

Keep up to date with the latest research on this topic with citation updates for this article.

Subscribe to citation updates

Read on this site (2)

Yiran Wang & Rickie Patani. (2020) Novel therapeutic targets for amyotrophic lateral sclerosis: ribonucleoproteins and cellular autonomy. Expert Opinion on Therapeutic Targets 24:10, pages 971-984.
Read now
Francesca Svetoni, Paola Frisone & Maria Paola Paronetto. (2016) Role of FET proteins in neurodegenerative disorders. RNA Biology 13:11, pages 1089-1102.
Read now

Articles from other publishers (61)

Alice Lippi & Anita Krisko. (2023) Protein aggregation: A detrimental symptom or an adaptation mechanism?. Journal of Neurochemistry.
Crossref
Korrie L. Mack, Hanna Kim, Edward M. Barbieri, JiaBei Lin, Sylvanne Braganza, Meredith E. Jackrel, Jamie E. DeNizio, Xiaohui Yan, Edward Chuang, Amber Tariq, Ryan R. Cupo, Laura M. Castellano, Kim A. Caldwell, Guy A. Caldwell & James Shorter. (2023) Tuning Hsp104 specificity to selectively detoxify α-synuclein. Molecular Cell 83:18, pages 3314-3332.e9.
Crossref
Florent Laval, Georges Coppin, Jean-Claude Twizere & Marc Vidal. (2023) Homo cerevisiae—Leveraging Yeast for Investigating Protein–Protein Interactions and Their Role in Human Disease. International Journal of Molecular Sciences 24:11, pages 9179.
Crossref
Matthew K. Howard, Karlie R. Miller, Brian S. Sohn, Jeremy J. Ryan, Andy Xu & Meredith E. Jackrel. (2023) Probing the drivers of Staphylococcus aureus biofilm protein amyloidogenesis and disrupting biofilms with engineered protein disaggregases . mBio.
Crossref
Emmanuel A. Ayeni, Ahmad M. Aldossary, Daniel A. Ayejoto, Lanre A. Gbadegesin, Abdullah A. Alshehri, Haya A. Alfassam, Henok K. Afewerky, Fahad A. Almughem, Saidu M. Bello & Essam A. Tawfik. (2022) Neurodegenerative Diseases: Implications of Environmental and Climatic Influences on Neurotransmitters and Neuronal Hormones Activities. International Journal of Environmental Research and Public Health 19:19, pages 12495.
Crossref
Amandeep Girdhar & Lin Guo. (2022) Regulating Phase Transition in Neurodegenerative Diseases by Nuclear Import Receptors. Biology 11:7, pages 1009.
Crossref
Maria S. Yurkova & Alexey N. Fedorov. (2022) GroEL—A Versatile Chaperone for Engineering and a Plethora of Applications. Biomolecules 12:5, pages 607.
Crossref
Macy L. Sprunger, Ken Lee, Brian S. Sohn & Meredith E. Jackrel. (2022) Molecular determinants and modifiers of Matrin-3 toxicity, condensate dynamics, and droplet morphology. iScience 25:3, pages 103900.
Crossref
Hana M. Odeh, Charlotte M. Fare & James Shorter. (2022) Nuclear-Import Receptors Counter Deleterious Phase Transitions in Neurodegenerative Disease. Journal of Molecular Biology 434:1, pages 167220.
Crossref
Jeremy J. Ryan, Aaron Bao, Braxton Bell, Cendi Ling & Meredith E. Jackrel. (2021) Drivers of Hsp104 potentiation revealed by scanning mutagenesis of the middle domain. Protein Science 30:8, pages 1667-1685.
Crossref
Macy L. Sprunger & Meredith E. Jackrel. (2021) Prion-Like Proteins in Phase Separation and Their Link to Disease. Biomolecules 11:7, pages 1014.
Crossref
Bede Portz, Bo Lim Lee & James Shorter. (2021) FUS and TDP-43 Phases in Health and Disease. Trends in Biochemical Sciences 46:7, pages 550-563.
Crossref
Charlotte M. Fare & James Shorter. (2021) (Dis)Solving the problem of aberrant protein states. Disease Models & Mechanisms 14:5.
Crossref
April L. Darling & James Shorter. (2021) Combating deleterious phase transitions in neurodegenerative disease. Biochimica et Biophysica Acta (BBA) - Molecular Cell Research 1868:5, pages 118984.
Crossref
Mrinal K. Poddar, Apala Chakraborty & Soumyabrata Banerjee. 2021. Oxidoreductase. Oxidoreductase.
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. (2020) Therapeutic genetic variation revealed in diverse Hsp104 homologs. eLife 9.
Crossref
Ritesh Kumar, Lisha Khungar, Rinchuila Shimphrui, Lalit Dev Tiwari, Gayatri Tripathi, Neelam K. Sarkar, Surekha-Katiyar Agarwal, Manu Agarwal & Anil Grover. (2020) AtHsp101 research sets course of action for the genetic improvement of crops against heat stress. Journal of Plant Biochemistry and Biotechnology 29:4, pages 715-732.
Crossref
Matthew K. Howard, Brian S. Sohn, Julius von Borcke, Andy Xu & Meredith E. Jackrel. (2020) Functional analysis of proposed substrate-binding residues of Hsp104. PLOS ONE 15:3, pages e0230198.
Crossref
Elizabeth A. Sweeny, Amber Tariq, Esin Gurpinar, Michelle S. Go, Matthew A. Sochor, Zhong-Yuan Kan, Leland Mayne, S. Walter Englander & James Shorter. (2020) Structural and mechanistic insights into Hsp104 function revealed by synchrotron X-ray footprinting. Journal of Biological Chemistry 295:6, pages 1517-1538.
Crossref
Xiufang Ding, Fude Sun, Jialin Chen, Long Chen, Yuto Tobin-Miyaji, Song Xue, Wei Qiang & Shi-Zhong Luo. (2020) Amyloid-Forming Segment Induces Aggregation of FUS-LC Domain from Phase Separation Modulated by Site-Specific Phosphorylation. Journal of Molecular Biology 432:2, pages 467-483.
Crossref
Yury O. Chernoff, Anastasia V. Grizel, Aleksandr A. Rubel, Andrew A. Zelinsky, Pavithra Chandramowlishwaran & Tatiana A. Chernova. 2020. 293 380 .
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. (2019) Mining Disaggregase Sequence Space to Safely Counter TDP-43, FUS, and α-Synuclein Proteotoxicity. Cell Reports 28:8, pages 2080-2095.e6.
Crossref
Jeremy J. Ryan, Macy L. Sprunger, Kayla Holthaus, James Shorter & Meredith E. Jackrel. (2019) Engineered protein disaggregases mitigate toxicity of aberrant prion-like fusion proteins underlying sarcoma. Journal of Biological Chemistry 294:29, pages 11286-11296.
Crossref
Clarissa L. Durie, JiaBei Lin, Nathaniel W. Scull, Korrie L. Mack, Meredith E. Jackrel, Elizabeth A. Sweeny, Laura M. Castellano, James Shorter & Aaron L. Lucius. (2019) Hsp104 and Potentiated Variants Can Operate as Distinct Nonprocessive Translocases. Biophysical Journal 116:10, pages 1856-1872.
Crossref
Zachary M. March, Korrie L. Mack & James Shorter. (2019) AAA+ Protein-Based Technologies to Counter Neurodegenerative Disease. Biophysical Journal 116:8, pages 1380-1385.
Crossref
Lucas C. Reineke & Joel R. Neilson. (2019) Differences between acute and chronic stress granules, and how these differences may impact function in human disease. Biochemical Pharmacology 162, pages 123-131.
Crossref
Veronica Verdile, Elisa De Paola & Maria Paola Paronetto. (2019) Aberrant Phase Transitions: Side Effects and Novel Therapeutic Strategies in Human Disease. Frontiers in Genetics 10.
Crossref
Karolina Michalska, Kaiming Zhang, Zachary M. March, Catherine Hatzos-Skintges, Grigore Pintilie, Lance Bigelow, Laura M. Castellano, Leann J. Miles, Meredith E. Jackrel, Edward Chuang, Robert Jedrzejczak, James Shorter, Wah Chiu & Andrzej Joachimiak. (2019) Structure of Calcarisporiella thermophila Hsp104 Disaggregase that Antagonizes Diverse Proteotoxic Misfolding Events. Structure 27:3, pages 449-463.e7.
Crossref
Zachary T. Monahan, Shannon N. Rhoads, Debra S. Yee & Frank P. Shewmaker. (2018) Yeast Models of Prion-Like Proteins That Cause Amyotrophic Lateral Sclerosis Reveal Pathogenic Mechanisms. Frontiers in Molecular Neuroscience 11.
Crossref
Rashad Hussain, Hira Zubair, Sarah Pursell & Muhammad Shahab. (2018) Neurodegenerative Diseases: Regenerative Mechanisms and Novel Therapeutic Approaches. Brain Sciences 8:9, pages 177.
Crossref
Amber Tariq, JiaBei Lin, Megan M Noll, Mariana P Torrente, Korrie L Mack, Oscar Hernandez Murillo, Meredith E Jackrel & James Shorter. (2018) Potentiating Hsp104 activity via phosphomimetic mutations in the middle domain. FEMS Yeast Research 18:5.
Crossref
Nadinath B. Nillegoda, Anne S. Wentink & Bernd Bukau. (2018) Protein Disaggregation in Multicellular Organisms. Trends in Biochemical Sciences 43:4, pages 285-300.
Crossref
Ronald Melki. (2018) How the shapes of seeds can influence pathology. Neurobiology of Disease 109, pages 201-208.
Crossref
Wouter Peelaerts, Veerle Baekelandt & Patrik Brundin. 2018. The Molecular and Cellular Basis of Neurodegenerative Diseases. The Molecular and Cellular Basis of Neurodegenerative Diseases 189 242 .
P. Wang, J. Li & B. Sha. (2018) Preliminary X-Ray Crystallographic Studies of the N-Terminal Domains of Hsp104 from Yeast Candida albicans and Saccharomyces cerevisiae. Crystallography Reports 62:7, pages 1171-1176.
Crossref
Zachary Monahan, Veronica H Ryan, Abigail M Janke, Kathleen A Burke, Shannon N Rhoads, Gül H Zerze, Robert O'Meally, Gregory L Dignon, Alexander E Conicella, Wenwei Zheng, Robert B Best, Robert N Cole, Jeetain Mittal, Frank Shewmaker & Nicolas L Fawzi. (2017) Phosphorylation of the FUS low‐complexity domain disrupts phase separation, aggregation, and toxicity . The EMBO Journal 36:20, pages 2951-2967.
Crossref
Destiny-Love Manecka, Benoît Vanderperre, Edward A. Fon & Thomas M. Durcan. (2017) The Neuroprotective Role of Protein Quality Control in Halting the Development of Alpha-Synuclein Pathology. Frontiers in Molecular Neuroscience 10.
Crossref
Lin Guo & James Shorter. (2017) Biology and Pathobiology of TDP-43 and Emergent Therapeutic Strategies. Cold Spring Harbor Perspectives in Medicine 7:9, pages a024554.
Crossref
Jeremy D. Baker, Lindsey B. Shelton, Dali Zheng, Filippo Favretto, Bryce A. Nordhues, April Darling, Leia E. Sullivan, Zheying Sun, Parth K. Solanki, Mackenzie D. Martin, Amirthaa Suntharalingam, Jonathan J. Sabbagh, Stefan Becker, Eckhard Mandelkow, Vladimir N. Uversky, Markus Zweckstetter, Chad A. Dickey, John Koren & Laura J. Blair. (2017) Human cyclophilin 40 unravels neurotoxic amyloids. PLOS Biology 15:6, pages e2001336.
Crossref
James Shorter. (2017) Designer protein disaggregases to counter neurodegenerative disease. Current Opinion in Genetics & Development 44, pages 1-8.
Crossref
Alice Ford HarrisonJames Shorter. (2017) RNA-binding proteins with prion-like domains in health and disease. Biochemical Journal 474:8, pages 1417-1438.
Crossref
Peng Wang, Jingzhi Li, Clarissa Weaver, Aaron Lucius & Bingdong Sha. (2017) Crystal structures of Hsp104 N-terminal domains from Saccharomyces cerevisiae and Candida albicans suggest the mechanism for the function of Hsp104 in dissolving prions . Acta Crystallographica Section D Structural Biology 73:4, pages 365-372.
Crossref
Meredith E. Jackrel & James Shorter. (2017) Protein-Remodeling Factors As Potential Therapeutics for Neurodegenerative Disease. Frontiers in Neuroscience 11.
Crossref
Tatiana A. Chernova, Keith D. Wilkinson & Yury O. Chernoff. (2017) Prions, Chaperones, and Proteostasis in Yeast. Cold Spring Harbor Perspectives in Biology 9:2, pages a023663.
Crossref
Sandeep K. Sharma & Smriti Priya. (2016) Expanding role of molecular chaperones in regulating ?-synuclein misfolding; implications in Parkinson?s disease. Cellular and Molecular Life Sciences 74:4, pages 617-629.
Crossref
Wouter Peelaerts & Tiago F. Outeiro. 2017. Disease-Modifying Targets in Neurodegenerative Disorders. Disease-Modifying Targets in Neurodegenerative Disorders 21 47 .
Ronald Melki. 2017. Disease-Modifying Targets in Neurodegenerative Disorders. Disease-Modifying Targets in Neurodegenerative Disorders 1 20 .
Zachary M. March, Oliver D. King & James Shorter. (2016) Prion-like domains as epigenetic regulators, scaffolds for subcellular organization, and drivers of neurodegenerative disease. Brain Research 1647, pages 9-18.
Crossref
Adriano Aguzzi & Matthias Altmeyer. (2016) Phase Separation: Linking Cellular Compartmentalization to Disease. Trends in Cell Biology 26:7, pages 547-558.
Crossref
James Shorter. (2016) Engineering therapeutic protein disaggregases. Molecular Biology of the Cell 27:10, pages 1556-1560.
Crossref
Elizabeth A. Sweeny & James Shorter. (2016) Mechanistic and Structural Insights into the Prion-Disaggregase Activity of Hsp104. Journal of Molecular Biology 428:9, pages 1870-1885.
Crossref
Korrie L. Mack & James Shorter. (2016) Engineering and Evolution of Molecular Chaperones and Protein Disaggregases with Enhanced Activity. Frontiers in Molecular Biosciences 3.
Crossref
Mariana P. Torrente, Edward Chuang, Megan M. Noll, Meredith E. Jackrel, Michelle S. Go & James Shorter. (2016) Mechanistic Insights into Hsp104 Potentiation. Journal of Biological Chemistry 291:10, pages 5101-5115.
Crossref
Wouter Hubens & Ayako Okado-Matsumoto. 2016. Redox-Active Therapeutics. Redox-Active Therapeutics 605 626 .
Meredith E. Jackrel, Keolamau Yee, Amber Tariq, Annie I. Chen & James Shorter. (2015) Disparate Mutations Confer Therapeutic Gain of Hsp104 Function. ACS Chemical Biology 10:12, pages 2672-2679.
Crossref
J.-J. Hauw, S. Ha?k & C. Duyckaerts. (2015) La transconformation prot?ique, nouveau paradigme en neurologie. Revue Neurologique 171:12, pages 825-831.
Crossref
Iris Lindberg, James Shorter, R. Luke Wiseman, Fabrizio Chiti, Chad A. Dickey & Pamela J. McLean. (2015) Chaperones in Neurodegeneration. The Journal of Neuroscience 35:41, pages 13853-13859.
Crossref
Lin Guo & James Shorter. (2015) It?s Raining Liquids: RNA Tunes Viscoelasticity and Dynamics of Membraneless Organelles. Molecular Cell 60:2, pages 189-192.
Crossref
Eva Asp, Ming Proschitsky & Rajaraman Krishnan. (2015) Remodeling Amyloid Fibers: Baker’s Yeast Shows Us the Way. Chemistry & Biology 22:8, pages 979-981.
Crossref
Laura M. Castellano, Stephen M. Bart, Veronica M. Holmes, Drew Weissman & James Shorter. (2015) Repurposing Hsp104 to Antagonize Seminal Amyloid and Counter HIV Infection. Chemistry & Biology 22:8, pages 1074-1086.
Crossref
Alejandra Aguado, José Angel Fernández-Higuero, Fernando Moro & Arturo Muga. (2015) Chaperone-assisted protein aggregate reactivation: Different solutions for the same problem. Archives of Biochemistry and Biophysics 580, pages 121-134.
Crossref

Related research

People also read lists articles that other readers of this article have read.

Recommended articles lists articles that we recommend and is powered by our AI driven recommendation engine.

Cited by lists all citing articles based on Crossref citations.
Articles with the Crossref icon will open in a new tab.