James Shorter

Also affiliated with California University of Pennsylvania, University of Pennsylvania

Biochemistry, Genetics and Molecular BiologyMedicine

Pilot corpus only

This score is computed over theSindex pilot corpus and does not cover the full scientific literature. Scores are relative to papers we have ingested — papers, authors, and institutions outside the pilot are not represented. Methodology.

Top 100%percentile

0Author DataRank

Indexed papers

0in pilot corpus

datarank_citation_only_1hop_v6· scope data_onlyMethodology

Papers

Driven by 53 papers. Top paper: “TDP-43 represses cryptic exon inclusion in the FTD–ALS gene UNC13A”.

TDP-43 represses cryptic exon inclusion in the FTD–ALS gene UNC13A

Nature(2022)10.1038/s41586-022-04424-7

N/A

0.957DataRank · unranked

589 citations

Mercedes Prudencio, Yuka Koike, Sarat C. Vatsavayai, Garam Kım, Fred Harbinski +30 more

Poly(ADP-ribose) drives condensation of FUS via a transient interaction

Molecular Cell(2022)10.1016/j.molcel.2022.01.018

N/A

0.663DataRank · unranked

82 citations

Morgan Dasovich, Joseph Yoniles, Mohsen Badiee, Sophie Skanchy, Laura R. Ganser +6 more

Characterization of HNRNPA1 mutations defines diversity in pathogenic mechanisms and clinical presentation

JCI Insight(2021)10.1172/jci.insight.148363

N/A

0.648DataRank · unranked

74 citations

Hong Joo Kim, Lin Guo, Kevin J. O’Donovan, Inès Mademan, Tine Deconinck +21 more

Heterozygous frameshift variants in HNRNPA2B1 cause early-onset oculopharyngeal muscular dystrophy

Nature Communications(2022)10.1038/s41467-022-30015-1

N/A

0.571DataRank · unranked

44 citations

Payam Mohassel, Sandra Donkervoort, Lin Guo, Kevin O’Donovan, Maura Coughlin +53 more

Opposing roles of p38α-mediated phosphorylation and PRMT1-mediated arginine methylation in driving TDP-43 proteinopathy

Cell Reports(2025)10.1016/j.celrep.2024.115205

N/A

0.425DataRank · unranked

16 citations

Hana M. Odeh, Heike J. Wobst, Bo Lim Lee, Úna Chan, Jocelyn C. Mauna +27 more

Cryo-EM Structure of the Full-length hnRNPA1 Amyloid Fibril

Journal of Molecular Biology(2023)10.1016/j.jmb.2023.168211

N/A

0.396DataRank · unranked

13 citations

Sambhasan Banerjee, Dilan Savran, Cedric Rajes, Sebastian Wiese, Amandeep Girdhar +7 more

NLS-binding deficient Kapβ2 reduces neurotoxicity via selective interaction with C9orf72-ALS/FTD dipeptide repeats

Communications Biology(2025)10.1038/s42003-024-07412-x

N/A

0.292DataRank · unranked

6 citations

Amandeep Girdhar, M.E. Cicardi, V. Kankate, Miyuki Hayashi, Ruoyu Yang +7 more

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

Journal of Clinical Investigation(2021)10.1172/jci149564

N/A

0.292DataRank · unranked

6 citations

Sebum Lee, Yulei Shang, Wenyuan Wang, Kin Fai Au, Sherry Kamiya +15 more

Heat Shock Protein Grp78/BiP/HspA5 Binds Directly to TDP-43 and Mitigates Toxicity Associated with Neurodegenerative Disease Pathology

Research Square(2021)10.21203/rs.3.rs-1090289/v1

N/A

0.104DataRank · unranked

1 citations

David D. Scott, Andrew J. Ambrose, Christopher J. Zerio, Kumara Dissanayake, Danielle G. May +10 more

Karyopherin-β2 Inhibits and Reverses Aggregation and Liquid-liquid Phase Separation of the ALS/FTD-Associated Protein FUS

BIO-PROTOCOL(2020)10.21769/bioprotoc.3725

N/A

0DataRank · unranked

3 citations

James Shorter, Lin Guo, Emma Robinson

Tuning Hsp104 specificity to selectively detoxify α-synuclein

bioRxiv (Cold Spring Harbor Laboratory)(2020)10.1101/2020.04.15.043935

N/A

0DataRank · unranked

4 citations

Hanna Kim, Meredith E. Jackrel, JiaBei Lin, Jamie E. DeNizio, Xiaohui Yan +8 more

Therapeutic genetic variation revealed in diverse Hsp104 homologs

bioRxiv (Cold Spring Harbor Laboratory)(2020)10.1101/2020.03.25.008748

N/A

0DataRank · unranked

2 citations

Katelyn Sweeney, Hanna Kim, Xiaohui Yan, Laura M. Castellano, Meredith E. Jackrel +10 more

Sequestration of TDP-43 216-414 aggregates by cytoplasmic expression of the proSAAS chaperone

bioRxiv (Cold Spring Harbor Laboratory)(2020)10.1101/2020.04.15.039578

N/A

0DataRank · unranked

2 citations

Kriti Chaplot, Timothy S. Jarvela, Edward M. Barbieri, James Shorter, Iris Lindberg +1 more

A PDZ-Binding Motif Controls Basolateral Targeting of Syndecan-1 Along the Biosynthetic Pathway in Polarized Epithelial Cells

UNC Libraries(2020)10.17615/stpz-j523

N/A

0DataRank · unranked

0 citations

Heike Fölsch, James M. Anderson, Jeff Sfakianos, Zenta Walther, Eric Anderson +5 more

Mechanistic Insights into Hsp104 Potentiation

UNC Libraries(2020)10.17615/nv10-1d45

N/A

0DataRank · unranked

0 citations

Michelle S. Go, James Shorter, Edward Chuang, Mariana P. Torrente, Megan M. Noll +1 more

Combating deleterious phase transitions in neurodegenerative disease

Biochimica et Biophysica Acta (BBA) - Molecular Cell Research(2021)10.1016/j.bbamcr.2021.118984

N/A

0DataRank · unranked

91 citations

James Shorter, April L. Darling

N-alpha-acetylation of Huntingtin protein increases its propensity to aggregate

Journal of Biological Chemistry(2021)10.1016/j.jbc.2021.101363

N/A

0DataRank · unranked

31 citations

Lin Guo, James Shorter, Ronen Marmorstein, Leah Gottlieb

(Dis)Solving the problem of aberrant protein states

Disease Models & Mechanisms(2021)10.1242/dmm.048983

N/A

0DataRank · unranked

30 citations

James Shorter, Charlotte M. Fare

Nuclear-Import Receptors Counter Deleterious Phase Transitions in Neurodegenerative Disease

Journal of Molecular Biology(2021)10.1016/j.jmb.2021.167220

N/A

0DataRank · unranked

21 citations

Charlotte M. Fare, James Shorter, Hana M. Odeh

Open Access: A Role for p53 in c9ALS/FTD?

Trends in Genetics(2021)10.1016/j.tig.2021.01.008

N/A

0DataRank · unranked

3 citations

James Shorter, Charlotte M. Fare

Specific heterozygous frameshift variants in hnRNPA2B1 cause early-onset oculopharyngeal muscular dystrophy

medRxiv(2021)10.1101/2021.04.08.21254942

N/A

0DataRank · unranked

2 citations

Payam Mohassel, Sandra Donkervoort, Lin Guo, Kevin J. O’Donovan, Maura Coughlin +53 more

DEFINING THE DIVERSITY OF HNRNPA1 MUTATIONS IN CLINICAL PHENOTYPE AND PATHOMECHANISM

medRxiv(2021)10.1101/2021.02.02.21250330

N/A

0DataRank · unranked

1 citations

Hyung Jun Kim, Lin Guo, Kevin J. O’Donovan, Inès Mademan, Tine Deconinck +21 more

Nuclear import receptors are recruited by FG-nucleoporins to rescue hallmarks of TDP-43 proteinopathy

Molecular Neurodegeneration(2022)10.1186/s13024-022-00585-1

N/A

0DataRank · unranked

48 citations

Deepak Chhangani, Melissa C. Wren, Courtney L. Smith, Jannifer H. Lee, Xingli Li +23 more

Heat shock protein Grp78/BiP/HspA5 binds directly to TDP-43 and mitigates toxicity associated with disease pathology

Scientific Reports(2022)10.1038/s41598-022-12191-8

N/A

0DataRank · unranked

41 citations

David D. Scott, Andrew J. Ambrose, Christopher J. Zerio, Marina Rodriguez-Sanchez, Kumara Dissanayake +11 more

Unique structural features govern the activity of a human mitochondrial AAA+ disaggregase, Skd3

Cell Reports(2022)10.1016/j.celrep.2022.111408

N/A

0DataRank · unranked

24 citations

Alexandrea N. Rizo, Gabriel A. Braun, Eric Tse, Edward Chuang, Kushol Gupta +3 more

Unique structural features govern the activity of a human mitochondrial AAA+ disaggregase, Skd3

bioRxiv (Cold Spring Harbor Laboratory)(2022)10.1101/2022.02.17.480866

N/A

0DataRank · unranked

4 citations

Alexandrea N. Rizo, Gabriel A. Braun, Eric Tse, Edward Chuang, Daniel R. Southworth +2 more

Increased Nuclear Localization of Engineered Hsp104 Variants Mitigates aS, FUS, and TDP‐43 Toxicity in Yeast

The FASEB Journal(2022)10.1096/fasebj.2022.36.s1.r2730

N/A

0DataRank · unranked

0 citations

James Shorter, JiaBei Lin, Benjamin Mass

Engineered Nuclear Import Receptor Karyopherin‐β2 Chaperones Aberrant Phase Transitions of Disease‐Associated Cargo

The FASEB Journal(2022)10.1096/fasebj.2022.36.s1.r2282

N/A

0DataRank · unranked

0 citations

Vladislav A. Korobeynikov, Kevin Rhine, Joey Yoniles, Sua Myong, Neil A. Shneider +2 more

Developing RNA Therapeutics for TDP‐43 Proteinopathy in ALS/FTD

The FASEB Journal(2022)10.1096/fasebj.2022.36.s1.r4365

N/A

0DataRank · unranked

0 citations

Ashleigh Smirnov, James Shorter, Katie E. Copley

Molecular determinants of Karyopherin-β2 chaperone and disaggregation activity

bioRxiv (Cold Spring Harbor Laboratory)(2022)10.1101/2022.09.14.508025

N/A

0DataRank · unranked

0 citations

Kevin Rhine, Andrew Lam, Sua Myong, James Shorter, Charlotte M. Fare

Phase Separation in Biology and Disease; Current Perspectives and Open Questions

Journal of Molecular Biology(2023)10.1016/j.jmb.2023.167971

N/A

0DataRank · unranked

88 citations

Shasha Chong, Jörg Gsponer, Liam J. Holt, Dragomir Milovanović, Diana M. Mitrea +17 more

Repetitive elements in aging and neurodegeneration

Trends in Genetics(2023)10.1016/j.tig.2023.02.008

N/A

0DataRank · unranked

66 citations

James Shorter, Katie E. Copley

Regulation of Biomolecular Condensates by Poly(ADP-ribose)

Chemical Reviews(2023)10.1021/acs.chemrev.2c00851

N/A

0DataRank · unranked

37 citations

Hana M. Odeh, James Shorter, Sua Myong, Kevin Rhine

Tuning Hsp104 specificity to selectively detoxify α-synuclein

Molecular Cell(2023)10.1016/j.molcel.2023.07.029

N/A

0DataRank · unranked

26 citations

Hanna Kim, Edward M. Barbieri, JiaBei Lin, Sylvanne Braganza, Meredith E. Jackrel +10 more

C-terminal frameshift variant of TDP-43 with pronounced aggregation-propensity causes rimmed vacuole myopathy but not ALS/FTD

Acta Neuropathologica(2023)10.1007/s00401-023-02565-1

N/A

0DataRank · unranked

24 citations

Nika Schuermans, Antoon Meylemans, Pontus LeBlanc, Lauren Versluys, Katie E. Copley +17 more

Nuclear-import receptors as gatekeepers of pathological phase transitions in ALS/FTD

Molecular Neurodegeneration(2024)10.1186/s13024-023-00698-1

N/A

0DataRank · unranked

31 citations

Miriam Linsenmeier, Courtney L. Smith, James Shorter, Wilfried Rossoll, Bilal Khalil

CRISPR screen for protein inclusion formation uncovers a role for SRRD in the regulation of intermediate filament dynamics and aggresome assembly

PLoS Genetics(2024)10.1371/journal.pgen.1011138

N/A

0DataRank · unranked

5 citations

Sapanna Chantarawong, Edward M. Barbieri, Greg Cajka, Matthew Liu, Lynn A. Spruce +11 more

The middle domain of Hsp104 can ensure substrates are functional after processing

PLoS Genetics(2024)10.1371/journal.pgen.1011424

N/A

0DataRank · unranked

5 citations

Jane E. Dorweiler, Sam Guereca, Brett T. Wisniewski, James Shorter, Anita L. Manogaran +1 more

Decoding Skd3 (Human CLPB): A Mitochondrial Protein Disaggregase Critical for Human Health

Israel Journal of Chemistry(2024)10.1002/ijch.202300153

N/A

0DataRank · unranked

4 citations

James Shorter, Ryan R. Cupo

Design principles to tailor Hsp104 therapeutics

Cell Reports(2024)10.1016/j.celrep.2024.115005

N/A

0DataRank · unranked

2 citations

Peter J. Carman, Craig W. Gambogi, Nathan M. Kendsersky, Edward Chuang, Stephanie N. Gates +5 more

Defining a small-molecule stimulator of the human Hsp70-disaggregase system with selectivity for DnaJB proteins

bioRxiv (Cold Spring Harbor Laboratory)(2024)10.1101/2024.01.11.575109

N/A

0DataRank · unranked

3 citations

Ryan R. Cupo, J. Bevan, Mikhaila Rice, Shuli Mao, Erica L. Gorenberg +6 more

Dependence of mitochondrial calcium signalling and dynamics on the disaggregase, CLPB

Nature Communications(2025)10.1038/s41467-025-57641-9

N/A

0DataRank · unranked

8 citations

Víctor Hugo Sánchez-Vázquez, Benjamín Cartes-Saavedra, Denis Vecellio Reane, Ryan R. Cupo, Hilda Carolina Delgado-De la Herrán +9 more

FUS Mislocalization Rewires a Cortical Gene Network to Drive Cognitive and Behavioral Impairment in ALS

medRxiv(2025)10.1101/2025.06.16.25329673

N/A

0DataRank · unranked

3 citations

Félicie Lorenc, Aurélie Bombardier, Claudia De Tapia, Stéphane Dieterle, Cláudio Gouveia Roque +17 more

Nuclear Import Receptors Directly Bind to Arginine-Rich Dipeptide Repeat Proteins and Suppress Their Pathological Interactions

Cell Reports(2020)10.1016/j.celrep.2020.108538

N/A

0DataRank · unranked

126 citations

Sinem Usluer, Benjamin Bourgeois, Francesca Simonetti, Hana M. Odeh, Charlotte M. Fare +9 more

Nuclear-import receptors remodel the dilute phase to suppress phase transitions of RNA-binding proteins with prion-like domains

bioRxiv (Cold Spring Harbor Laboratory)(2025)10.1101/2025.11.14.688546

N/A

0DataRank · unranked

0 citations

Min Kyung Shinn, Thomas R. Mumford, Vicky Liu, Lukasz J. Bugaj, Rohit V. Pappu +2 more

ALS/FTLD-Linked Mutations in FUS Glycine Residues Cause Accelerated Gelation and Reduced Interactions with Wild-Type FUS

Molecular Cell(2020)10.1016/j.molcel.2020.10.014

N/A

0DataRank · unranked

97 citations

Monika A. Makurath, James Liu, Sophie Skanchy, Christian Lopez, Kevin F. Catalan +7 more

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

eLife(2020)10.7554/elife.55279

N/A

0DataRank · unranked

76 citations

James Shorter, Ryan R. Cupo

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

Cell Reports(2020)10.1016/j.celrep.2020.108418

N/A

0DataRank · unranked

65 citations

Dilshan S. Harischandra, Shivani Ghaisas, Pengfei Zhang, Wil Prall, Liangqian Huang +9 more

Atomic Structures of Amyloid-β Oligomers Illuminate a Neurotoxic Mechanism

Trends in Neurosciences(2020)10.1016/j.tins.2020.07.006

N/A

0DataRank · unranked

19 citations

James Shorter, April L. Darling

Just Took a DNA Test, Turns Out 100% Not That Phase

Molecular Cell(2020)10.1016/j.molcel.2020.03.029

N/A

0DataRank · unranked

12 citations

James Shorter, Taekjip Ha, Sua Myong, Aaron D. Gitler

Biochemical Timekeeping Via Reentrant Phase Transitions

Journal of Molecular Biology(2020)10.1016/j.jmb.2020.166794

N/A

0DataRank · unranked

41 citations

James Shorter, Bede Portz

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

Proceedings of the National Academy of Sciences(2020)10.1073/pnas.1921968117

N/A

0DataRank · unranked

33 citations

JiaBei Lin, Leland Mayne, James Shorter, S. Walter Englander, Xiang Ye

Therapeutic genetic variation revealed in diverse Hsp104 homologs

eLife(2020)10.7554/elife.57457

N/A

0DataRank · unranked

29 citations

Katelyn Sweeney, Hanna Kim, Xiaohui Yan, Laura M. Castellano, Meredith E. Jackrel +12 more