Publications by authors named "Jonathan D Cooper"

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Enteric nervous system degeneration in human and murine CLN3 disease, is ameliorated by gene therapy in mice.
Ewa A Ziółkowska, Letitia L Williams, Matthew J Jansen, Sophie H Wang, Elizabeth M Eultgen, Jonathan D Cooper

Acta Neuropathol Commun· December 2025

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Reduced heparan sulfate levels in cerebrospinal fluid reflect brain neuron correction in Sanfilippo B mice.
Steven Q Le, Alexander Sorensen, Soila Sukupolvi, Gianna Jewhurst, Grant Austin, Jonathan D Cooper

J Clin Invest· November 2025

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Neuronal ceroid lipofuscinosis: underlying mechanisms and emerging therapeutic targets.
Ewa A Ziółkowska, Keigo Takahashi, Patricia I Dickson, Marco Sardiello, Mark S Sands, Jonathan D Cooper

Nat Rev Neurol· November 2025

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GABAergic interneurons contribute to the fatal seizure phenotype of CLN2 disease mice.
Keigo Takahashi, Nicholas R Rensing, Elizabeth M Eultgen, Letitia L Williams, Sophie H Wang, Jonathan D Cooper

JCI Insight· October 2025

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Immunostaining Methods for Lysosomal Storage Disorder Research.
Ewa A Ziółkowska, Sophie H Wang, Hemanth R Nelvagal, Jonathan D Cooper

Methods Mol Biol· October 2025

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Enteric nervous system disease in neuronopathic lysosomal storage disorders.
Ewa A Ziółkowska, Robert O Heuckeroth, Jonathan D Cooper

Neural Regen Res· August 2025

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Gene therapy ameliorates neuromuscular pathology in CLN3 disease.
Ewa A Ziółkowska, Albina Jablonka-Shariff, Letitia L Williams, Matthew J Jansen, Sophie H Wang, Jonathan D Cooper

Acta Neuropathol Commun· July 2025

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Enteric nervous system degeneration in human and murine CLN3 disease, is ameliorated by gene therapy in mice.
Ewa A Ziółkowska, Letitia L Williams, Matthew J Jansen, Sophie H Wang, Elizabeth M Eultgen, Jonathan D Cooper

bioRxiv· June 2025

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Gene therapy ameliorates bowel dysmotility and enteric neuron degeneration and extends survival in lysosomal storage disorder mouse models.
Ewa A Ziółkowska, Matthew J Jansen, Letitia L Williams, Sophie H Wang, Elizabeth M Eultgen, Jonathan D Cooper

Sci Transl Med· January 2025

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Cellular trafficking and fate mapping of cells within the nervous system after in utero hematopoietic cell transplantation.
Matthew T Grant, Hemanth Ramesh Nelvagal, Maria Tecos, Amal Hamed, Kerry Swanson, Jonathan D Cooper

Commun Biol· December 2024

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GABAergic interneurons contribute to the fatal seizure phenotype of CLN2 disease mice.
Keigo Takahashi, Nicholas R Rensing, Elizabeth M Eultgen, Sophie H Wang, Hemanth R Nelvagal, Jonathan D Cooper

bioRxiv· March 2024

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Neuronal deletion of the circadian clock gene Bmal1 induces cell-autonomous dopaminergic neurodegeneration.
Michael F Kanan, Patrick W Sheehan, Jessica N Haines, Pedro G Gomez, Adya Dhuler, Jonathan D Cooper

JCI Insight· January 2024

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Severe central nervous system demyelination in Sanfilippo disease.
Mahsa Taherzadeh, Erjun Zhang, Irene Londono, Benjamin De Leener, Sophie Wang, Jonathan D Cooper

Front Mol Neurosci· December 2023

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A mouse mutant deficient in both neuronal ceroid lipofuscinosis-associated proteins CLN3 and TPP1.
David E Sleat, Whitney Banach-Petrosky, Katherine E Larrimore, Yuliya Nemtsova, Jennifer A Wiseman, Jonathan D Cooper

J Inherit Metab Dis· July 2023

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Gene therapy ameliorates spontaneous seizures associated with cortical neuron loss in a Cln2R207X mouse model.
Keigo Takahashi, Elizabeth M Eultgen, Sophie H Wang, Nicholas R Rensing, Hemanth R Nelvagal, Jonathan D Cooper

J Clin Invest· June 2023

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Brain transplantation of genetically corrected Sanfilippo type B neural stem cells induces partial cross-correction of the disease.
Yewande Pearse, Don Clarke, Shih-Hsin Kan, Steven Q Le, Valentina Sanghez, Jonathan D Cooper

Mol Ther Methods Clin Dev· December 2022

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Brain volume in chronic ketamine users - relationship to sub-threshold psychotic symptoms and relevance to schizophrenia.
Robert A Chesters, Fiona Pepper, Celia Morgan, Jonathan D Cooper, Oliver D Howes

Psychopharmacology (Berl)· November 2022

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Top-down and bottom-up propagation of disease in the neuronal ceroid lipofuscinoses.
John R Ostergaard, Hemanth R Nelvagal, Jonathan D Cooper

Front Neurol· November 2022

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Cross-species efficacy of enzyme replacement therapy for CLN1 disease in mice and sheep.
Hemanth R Nelvagal, Samantha L Eaton, Sophie H Wang, Elizabeth M Eultgen, Keigo Takahashi, Jonathan D Cooper

J Clin Invest· October 2022

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Tralesinidase Alfa Enzyme Replacement Therapy Prevents Disease Manifestations in a Canine Model of Mucopolysaccharidosis Type IIIB.
N Matthew Ellinwood, Bethann N Valentine, Andrew S Hess, Jackie K Jens, Elizabeth M Snella, Jonathan D Cooper

J Pharmacol Exp Ther· September 2022

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Modelling Neurological Diseases in Large Animals: Criteria for Model Selection and Clinical Assessment.
Samantha L Eaton, Fraser Murdoch, Nina M Rzechorzek, Gerard Thompson, Claudia Hartley, Jonathan D Cooper

Cells· August 2022

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Effects of chronic cannabidiol in a mouse model of naturally occurring neuroinflammation, neurodegeneration, and spontaneous seizures.
Joshua T Dearborn, Hemanth R Nelvagal, Nicholas R Rensing, Keigo Takahashi, Stephanie M Hughes, Jonathan D Cooper

Sci Rep· July 2022

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Glial Dysfunction and Its Contribution to the Pathogenesis of the Neuronal Ceroid Lipofuscinoses.
Keigo Takahashi, Hemanth R Nelvagal, Jenny Lange, Jonathan D Cooper

Front Neurol· April 2022

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Neuropathology of murine Sanfilippo D syndrome.
Keigo Takahashi, Steven Q Le, Shih-Hsin Kan, Matthew J Jansen, Patricia I Dickson, Jonathan D Cooper

Mol Genet Metab· December 2021

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Biochemical evaluation of intracerebroventricular rhNAGLU-IGF2 enzyme replacement therapy in neonatal mice with Sanfilippo B syndrome.
Shih-Hsin Kan, Ibrahim Elsharkawi, Steven Q Le, Heather Prill, Linley Mangini, Jonathan D Cooper

Mol Genet Metab· June 2021

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