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J

James Shorter

University of Pennsylvania

80H-index
285Paper Count
2.7WCitation Count
Published Papers 147
Publication Date
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Hypoxia rescues complex 1-associated disease caused by proteostatic defects
errNature Metabolism
IF20.8
err2026-07-08
err0
errOAAI
errAnkur Garg; Brandon R. Desousa; Raju Roy; Amy Flis; Skyler Y. Blume; Yohei Abe; Arthur A. Melo; Ryan R. Cupo; Gabriela Grigorean; Daniel R. Southworth; James Shorter; Isha H. Jain
errShare
errSave
Isoform-specific steric zippers drive aberrant assembly and mislocalization of shortened TDP-43
errScience Advances
IF12.5
err2026-06-24
err0
errOAAI
errKatie E. Copley; Megan M. Dykstra; Morgan R. Miller; Miriam Linsenmeier; Longsheng Lai; Yuanhang Wang; Yi-Wei Chang; Sami J. Barmada; James Shorter
errShare
errSave
Short RNA chaperones promote aggregation-resistant TDP-43 conformers to mitigate neurodegeneration
errScience
IF45.8
err2026-05-07
err0
PREAI
errKatie E. Copley; Jocelyn C. Mauna; Helen L. Danielson; Qizan Chen; Busra Ozguney; Marilyn Ngo; Longxin Xie; Ashleigh Smirnov; Matt Davis; Leland Mayne; Miriam Linsenmeier; Jack D. Rubien; Cristian A. Bergmann; Bede Portz; Bo Lim Lee; Hana M. Odeh; Longsheng Lai; Yi-Wei Chang; Martina Hallegger; Jernej Ule; Piera Pasinelli; Yan Poon; Jeetain Mittal; Nicolas L. Fawzi; Ben E. Black; Christopher J. Donnelly; Brigid K. Jensen; James Shorter
errShare
errSave
NLS-binding deficient Kapβ2 reduces neurotoxicity via selective interaction with C9orf72-ALS/FTD dipeptide repeats
errCOMMUNICATIONS BIOLOGY
IF5.1
err2025-01-02
err0
errOAAI
errKim, Kevin M.; Girdhar, Amandeep; Cicardi, Maria E.; Kankate, Vaishnavi; Hayashi, Miyuki; Yang, Ruoyu; Carey, Jenny L.; Fare, Charlotte M.; Shorter, James; Cingolani, Gino; Trotti, Davide; Guo, Lin
errShare
errSave
Opposing roles of p38α-mediated phosphorylation and PRMT1-mediated arginine methylation in driving TDP-43 proteinopathy
errCELL REPORTS
IF6.9
err2025-01-01
err2
errOAAI
errAikio, Mari; Odeh, Hana M.; Wobst, Heike J.; Lee, Bo Lim; Chan, Una; Mauna, Jocelyn C.; Mack, Korrie L.; Class, Bradley; Ollerhead, Thomas A.; Ford, Alice F.; Barbieri, Edward M.; Cupo, Ryan R.; Drake, Lauren E.; Smalley, Joshua L.; Lin, Yuan-Ta; Lam, Stephanie; Thomas, Reuben; Castello, Nicholas; Baral, Ashmita; Beyer, Jenna N.; Najar, Mohd A.; Dunlop, John; Gitler, Aaron D.; Javaherian, Ashkan; Kaye, Julia A.; Burslem, George M.; Brown, Dean G.; Donnelly, Christopher J.; Finkbeiner, Steven; Moss, Stephen J.; Brandon, Nicholas J.; Shorter, James
errShare
errSave
The middle domain of Hsp104 can ensure substrates are functional after processing
errPLOS GENETICS
IF3.7
err2024-10-03
err1
errOAAI
errBuchholz, Hannah E.; Dorweiler, Jane E.; Guereca, Sam; Wisniewski, Brett T.; Shorter, James; Manogaran, Anita L.
errShare
errSave
Frontotemporal dementia-like disease progression elicited by seeded aggregation and spread of FUS
errMOLECULAR NEURODEGENERATION
IF17.5
err2024-06-11
err0
errOAAI
errVazquez-Sanchez, Sonia; Tilkin, Britt; Gasset-Rosa, Fatima; Zhang, Sitao; Piol, Diana; McAlonis-Downes, Melissa; Artates, Jonathan; Govea-Perez, Noe; Verresen, Yana; Guo, Lin; Cleveland, Don W.; Shorter, James; Da Cruz, Sandrine
errShare
errSave
CRISPR screen for protein inclusion formation uncovers a role for SRRD in the regulation of intermediate filament dynamics and aggresome assembly
errPLOS GENETICS
IF3.7
err2024-02-05
err1
errOAAI
errSweeney, Katelyn M.; Chantarawong, Sapanna; Barbieri, Edward M.; Cajka, Greg; Liu, Matthew; Spruce, Lynn; Fazelinia, Hossein; Portz, Bede; Copley, Katie; Lapidot, Tomer; Duhamel, Lauren; Greenwald, Phoebe; Saida, Naseeb; Shalgi, Reut; Shorter, James; Shalem, Ophir
errShare
errSave
Nuclear-import receptors as gatekeepers of pathological phase transitions in ALS/FTD
errMOLECULAR NEURODEGENERATION
IF17.5
err2024-01-22
err9
errOAAI
errKhalil, Bilal; Linsenmeier, Miriam; Smith, Courtney L.; Shorter, James; Rossoll, Wilfried
errShare
errSave
Mo' m1A, mo' problems
errCELL CHEMICAL BIOLOGY
IF7.2
err2024-01-01
err0
errOAAI
errMiller, Linamarie; Shorter, James
errShare
errSave
Tuning Hsp104 specificity to selectively detoxify a-synuclein
errMOLECULAR CELL
IF16.6
err2023-09-01
err4
errOAAI
errMack, Korrie L.; Kim, Hanna; Barbieri, Edward M.; Lin, Jiabei; Braganza, Sylvanne; Jackrel, Meredith E.; Denizio, Jamie E.; Yan, Xiaohui; Chuang, Edward; Tariq, Amber; Cupo, Ryan R.; Castellano, Laura M.; Caldwell, Kim A.; Caldwell, Guy A.; Shorter, James
errShare
errSave
Transcriptome-wide RNA binding analysis of C9orf72 poly(PR) dipeptides
errLIFE SCIENCE ALLIANCE
IF2.9
err2023-07-12
err6
errOAAI
errBalendra, Rubika; de los Mozos, Igor Ruiz; Odeh, Hana M.; Glaria, Idoia; Milioto, Carmelo; Wilson, Katherine M.; Ule, Agnieszka M.; Hallegger, Martina; Masino, Laura; Martin, Stephen; Patani, Rickie; Shorter, James; Ule, Jernej; Isaacs, Adrian M.
errShare
errSave
Repetitive elements in aging and neurodegeneration
errTRENDS IN GENETICS
IF16.3
err2023-05-01
err18
errOAAI
errCopley, Katie E.; Shorter, James
errShare
errSave
C-terminal frameshift variant of TDP-43 with pronounced aggregation-propensity causes rimmed vacuole myopathy but not ALS/FTD
errACTA NEUROPATHOLOGICA
IF9.3
err2023-03-31
err12
errOAAI
errErvilha Pereira, Pedro; Schuermans, Nika; Meylemans, Antoon; LeBlanc, Pontus; Versluys, Lauren; Copley, Katie E. E.; Rubien, Jack D. D.; Altheimer, Christopher; Peetermans, Myra; Debackere, Elke; Vanakker, Olivier; Janssens, Sandra; Baets, Jonathan; Verhoeven, Kristof; Lammens, Martin; Symoens, Sofie; De Paepe, Boel; Barmada, Sami J. J.; Shorter, James; De Bleecker, Jan L. L.; Bogaert, Elke; Dermaut, Bart
errShare
errSave
A minimal construct of nuclear-import receptor Karyopherin-β2 defines the regions critical for chaperone and disaggregation activity
errJOURNAL OF BIOLOGICAL CHEMISTRY
IF3.9
err2023-02-01
err10
errOAAI
errFare, Charlotte M.; Rhine, Kevin; Lam, Andrew; Myong, Sua; Shorter, James
errShare
errSave
Nuclear import receptors are recruited by FG-nucleoporins to rescue hallmarks of TDP-43 proteinopathy
errMOLECULAR NEURODEGENERATION
IF17.5
err2022-12-08
err17
errOAAI
errKhalil, Bilal; Chhangani, Deepak; Wren, Melissa C.; Smith, Courtney L.; Lee, Jannifer H.; Li, Xingli; Puttinger, Christian; Tsai, Chih-Wei; Fortin, Gael; Morderer, Dmytro; Gao, Junli; Liu, Feilin; Lim, Chun Kim; Chen, Jingjiao; Chou, Ching-Chieh; Croft, Cara L.; Gleixner, Amanda M.; Donnelly, Christopher J.; Golde, Todd E.; Petrucelli, Leonard; Oskarsson, Bjorn; Dickson, Dennis W.; Zhang, Ke; Shorter, James; Yoshimura, Shige H.; Barmada, Sami J.; Rincon-Limas, Diego E.; Rossoll, Wilfried
errShare
errSave
Advanced Molecular Tweezers with Lipid Anchors against SARS-CoV-2 and Other Respiratory Viruses
errJACS AU
IF8.7
err2022-09-06
err5
errOAAI
errWeil, Tatjana; Kirupakaran, Abbna; Le, My-Hue; Rebmann, Philipp; Mieres-Perez, Joel; Issmail, Leila; Conzelmann, Carina; Mueller, Janis A.; Rauch, Lena; Gilg, Andrea; Wettstein, Lukas; Gross, Ruediger; Read, Clarissa; Bergner, Tim; Palsson, Sandra Axberg; Uhlig, Nadja; Eberlein, Valentina; Woell, Heike; Klaerner, Frank-Gerrit; Stenger, Steffen; Kuemmerer, Beate M.; Streeck, Hendrik; Fois, Giorgio; Frick, Manfred; Braubach, Peter; Spetz, Anna-Lena; Grunwald, Thomas; Shorter, James; Sanchez-Garcia, Elsa; Schrader, Thomas; Muench, Jan
errShare
errSave
Unique structural features govern the activity of a human mitochondrial AAA plus disaggregase, Skd3
errCELL REPORTS
IF6.9
err2022-09-01
err9
errOAAI
errCupo, Ryan R.; Rizo, Alexandrea N.; Braun, Gabriel A.; Tse, Eric; Chuang, Edward; Gupta, Kushol; Southworth, Daniel R.; Shorter, James
errShare
errSave
Sexually dimorphic RNA helicases DDX3X and DDX3Y differentially regulate RNA metabolism through phase separation
errMOLECULAR CELL
IF16.6
err2022-07-01
err44
errOAAI
errShen, Hui; Yanas, Amber; Owens, Michael C.; Zhang, Celia; Fritsch, Clark; Fare, Charlotte M.; Copley, Katie E.; Shorter, James; Goldman, Yale E.; Liu, Kathy Fange
errShare
errSave
Heat shock protein Grp78/BiP/HspA5 binds directly to TDP-43 and mitigates toxicity associated with disease pathology
errSCIENTIFIC REPORTS
IF3.9
err2022-05-17
err20
errOAAI
errFrancois-Moutal, Liberty; Scott, David Donald; Ambrose, Andrew J.; Zerio, Christopher J.; Rodriguez-Sanchez, Marina; Dissanayake, Kumara; May, Danielle G.; Carlson, Jacob M.; Barbieri, Edward; Moutal, Aubin; Roux, Kyle J.; Shorter, James; Khanna, Rajesh; Barmada, Sami J.; McGurk, Leeanne; Khanna, May
errShare
errSave

Research Directions

No research directions
Co-authors
Cooperation Journals
J
John Q. Trojanowski
H-index: 255 · Papers: 1.8K
T
Trevor W. Robbins
H-index: 242 · Papers: 4.1K
D
Dennis W. Dickson
H-index: 218 · Papers: 2.3K
V
Virginia M.‐Y. Lee
H-index: 200 · Papers: 919
S
Stephen L. Buchwald
H-index: 177 · Papers: 1.3K
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