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J

Jonathan D. Cooper

washington university

91H-index
505Paper Count
2.4WCitation Count
Published Papers 145
Publication Date
Systemic AAV9 Gene Therapy Mitigates Neuromuscular Junction Degeneration and Muscle Atrophy in a Mouse Model of CLN1 Disease
err2026-03-31
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errEwa A. Ziółkowska; Albina Jablonka-Shariff; Letitia L. Williams; Elizabeth M. Eultgen; Matthew D. Wood; Daniel A. Hunter; Mark S. Sands; Alison K. Snyder-Warwick; Jonathan D. Cooper
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Recommendations for the diagnosis and management of cln3 disease (batten disease) using the Delphi consensus methodology
err2026-03-10
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errJonathan W. Mink; Heather R. Adams; Rebecca Ahrens-Nicklas; Brian Nauheimer Andersen; Erika Augustine; Rose‑Mary Boustany; Jonathan D. Cooper; Alex Levin; Paul Gissen; Minna Laine; Heather L. Mason; Sara E. Mole; Miriam Nickel; John R. Ostergaard; Lori Sikorra; Lauren Treat; Ineka T. Whiteman; Ruth Williams; Angela Schulz
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Neuropathological alterations in TPP1-deficient cynomolgus macaques recapitulate key features of human CLN2 disease
err2026-02-03
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PREAI
errEwa A. Ziolkowska; Sophie H. Wang; Grace Kick; Keigo Takahashi; Jonathan D. Cooper
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Limited therapeutic efficacy of N-acetyl-L-leucine in a mouse model of CLN1 disease
err2025-12-29
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errEwa A. Ziółkowska; Nicole A. Pagán Torres; Hsintsung Chen; Letitia L. Williams; Elizabeth M. Eultgen; Agnieszka Nowacka; Joshua T. Dearborn; Hemanth R. Nelvagal; Ineka T. Whiteman; Frances M. Platt; Jonathan D. Cooper
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Enteric nervous system degeneration in human and murine CLN3 disease, is ameliorated by gene therapy in mice
err2025-12-22
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errEwa A. Ziółkowska; Letitia L. Williams; Matthew J. Jansen; Sophie H. Wang; Elizabeth M. Eultgen; Jaiprakash Sharma; Marco Sardiello; Rebecca P. Bradley; Ineka T. Whiteman; Mark S. Sands; Robert O. Heuckeroth; Jonathan D. Cooper
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Reduced heparan sulfate levels in cerebrospinal fluid reflect brain neuron correction in Sanfilippo B mice
err2025-11-17
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errLe, Steven Q.; Sorensen, Alexander; Sukupolvi, Soila; Jewhurst, Gianna; Austin, Grant; Doray, Balraj; Cooper, Jonathan D.; Dickson, Patricia I.
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GABAergic interneurons contribute to the fatal seizure phenotype of CLN2 disease mice
err2025-10-08
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errTakahashi, Keigo; Rensing, Nicholas R.; Eultgen, Elizabeth M.; Williams, Letitia L.; Wang, Sophie H.; Nelvagal, Hemanth R.; Le, Steven Q.; Roberts, Marie S.; Doray, Balraj; Han, Edward B.; Dickson, Patricia I.; Wong, Michael; Sands, Mark S.; Cooper, Jonathan D.
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Treatment of dysphagia associated pathologies in CLN3 deficient mice via gene therapy
err2025-02-01
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PREAI
errZiolkowska, Ewa A.; Williams, Letitia L.; Eultgen, Elizabeth M.; Jablonka-Shariff, Albina; Nelvagal, Hemanth R.; Takahashi, Keigo; Snyder-Warwick, Alison; Heuckeroth, Robert O.; Cooper, Jonathan D.
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Cellular trafficking and fate mapping of cells within the nervous system after in utero hematopoietic cell transplantation
err2024-12-05
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errGrant, Matthew T.; Nelvagal, Hemanth Ramesh; Tecos, Maria; Hamed, Amal; Swanson, Kerry; Cooper, Jonathan D.; Vrecenak, Jesse D.
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Neuronal deletion of the circadian clock gene Bmal1 induces cell-autonomous dopaminergic neurodegeneration
err2024-01-23
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errKanan, Michael K.; Sheehan, Patrick W.; Haines, Jessica N.; Gomez, Pedro G.; Dhuler, Adya; Nadarajah, Collin J.; Wargel, Zachary M.; Freeberg, Brittany M.; Nelvagal, Hemanth R.; Izumo, Mariko; Takahashi, Joseph S.; Cooper, Jonathan D.; Davis, Albert A.; Musiek, Erik S.
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Severe central nervous system demyelination in Sanfilippo disease
err2023-12-13
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errTaherzadeh, Mahsa; Zhang, Erjun; Londono, Irene; De Leener, Benjamin; Wang, Sophie; Cooper, Jonathan D.; Kennedy, Timothy E.; Morales, Carlos R.; Chen, Zesheng; Lodygensky, Gregory A.; Pshezhetsky, Alexey V.
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Gene therapy ameliorates spontaneous seizures associated with cortical neuron loss in a Cln2R207X mouse model
err2023-06-15
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errTakahashi, Keigo; Eultgen, Elizabeth M.; Wang, Sophie H.; Rensing, Nicholas R.; Nelvagal, Hemanth R.; Dearborn, Joshua T.; Danos, Olivier; Buss, Nicholas; Sands, Mark S.; Wong, Michael; Cooper, Jonathan D.
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Spontaneous seizures associated with cortical interneuron loss in Cln2R207X mice are ameliorated via gene therapy
err2023-02-01
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PREAI
errTakahashi, Keigo; Eultgen, Elizabeth M.; Wang, Sophie H.; Rensing, Nicholas; Nelvagal, Hemanth R.; Dearborn, Joshua T.; Danos, Olivier; Buss, Nicholas; Sands, Mark; Wong, Michael; Cooper, Jonathan D.
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Cross-species efficacy of enzyme replacement therapy for CLN1 disease in mice and sheep
err2022-10-17
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errNelvagal, Hemanth R.; Eaton, Samantha L.; Wang, Sophie H.; Eultgen, Elizabeth M.; Takahashi, Keigo; Le, Steven Q.; Nesbitt, Rachel; Dearborn, Joshua T.; Siano, Nicholas; Puhl, Ana C.; Dickson, Patricia, I; Thompson, Gerard; Murdoch, Fraser; Brennan, Paul M.; Gray, Mark; Greenhalgh, Stephen N.; Tennant, Peter; Gregson, Rachael; Clutton, Eddie; Nixon, James; Proudfoot, Chris; Guido, Stefano; Lillico, Simon G.; Whitelaw, C. Bruce A.; Lu, Jui-Yun; Hofmann, Sandra L.; Ekins, Sean; Sands, Mark S.; Wishart, Thomas M.; Cooper, Jonathan D.
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Tralesinidase Alfa Enzyme Replacement Therapy Prevents Disease Manifestations in a Canine Model of Mucopolysaccharidosis Type IIIB
err2022-09-01
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errEllinwood, N. Matthew; Valentine, Bethann N.; Hess, Andrew S.; Jens, Jackie K.; Snella, Elizabeth M.; Jamil, Maryam; Hostetter, Shannon J.; Jeffery, Nicholas D.; Smith, Jodi D.; Millman, Suzanne T.; Parsons, Rebecca L.; Butt, Mark T.; Chandra, Sundeep; Egeland, Martin T.; Assis, Ana B.; Nelvagal, Hemanth R.; Cooper, Jonathan D.; Nestrasil, Igor; Mueller, Bryon A.; Labounek, Rene; Paulson, Amy; Prill, Heather; Liu, Xiao Ying; Zhou, Huiyu; Lawrence, Roger; Crawford, Brett E.; Grover, Anita; Cherala, Ganesh; Melton, Andrew C.; Cherukuri, Anu; Vuillemenot, Brian R.; Wait, Jill C. M.; O'Neill, Charles A.; Pinkstaff, Jason; Kovalchin, Joseph; Zanelli, Eric; McCullagh, Emma
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Modelling Neurological Diseases in Large Animals: Criteria for Model Selection and Clinical Assessment
errCELLS
IF5.2
err2022-08-25
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errEaton, Samantha L.; Murdoch, Fraser; Rzechorzek, Nina M.; Thompson, Gerard; Hartley, Claudia; Blacklock, Benjamin Thomas; Proudfoot, Chris; Lillico, Simon G.; Tennant, Peter; Ritchie, Adrian; Nixon, James; Brennan, Paul M.; Guido, Stefano; Mitchell, Nadia L.; Palmer, David N.; Whitelaw, C. Bruce A.; Cooper, Jonathan D.; Wishart, Thomas M.
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Effects of chronic cannabidiol in a mouse model of naturally occurring neuroinflammation, neurodegeneration, and spontaneous seizures
err2022-07-04
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errDearborn, Joshua T.; Nelvagal, Hemanth R.; Rensing, Nicholas R.; Takahashi, Keigo; Hughes, Stephanie M.; Wishart, Thomas M.; Cooper, Jonathan D.; Wong, Michael; Sands, Mark S.
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Amelioration of enteric nervous system defects via gene therapy in CLN1 disease mice
err2022-02-01
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PREAI
errCooper, Jonathan D.; Wang, Sophie H.; Williams, Letitia L.; Eultgen, Elizabeth M.; Takahashi, Keigo; Nelvagal, Hemanth R.; Anderson, Jessica B.; Makita, Takako; DeBosch, Brian; Sands, Mark S.; Heuckeroth, Robert O.
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Spinal cord pathology in murine Sanfilippo syndrome type B
err2022-02-01
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PREAI
errLe, Steven Q.; Sorensen, Alexander; Chintalapati, Keerthana; Kan, Shih-Hsin; Nelvagal, Hemanth R.; Cooper, Jonathan D.; Dickson, Patricia I.
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Efficacy of recombinant human PPT1 enzyme replacement therapy in mouse and sheep models of CLN1 disease
err2022-02-01
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PREAI
errNelvagal, Hemanth R.; Eaton, Samantha L.; Wang, Sophie H.; Eultgen, Elizabeth M.; Takahashi, Keigo; Le, Steven Q.; Nesbitt, LaRachel; Dearborn, Joshua T.; Puhl, Ana C.; Dickson, Patricia I.; Brennan, Paul M.; Thompson, Gerard; Hofmann, Sandra L.; Ekins, Sean; Sands, Mark S.; Wishart, Thomas M.; Cooper, Jonathan D.
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