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

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

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Combined Anti-inflammatory and Neuroprotective Treatments Have the Potential to Impact Disease Phenotypes in Cln3−/− Mice von Marta A. Tarczyluk-Wells, Christoph Salzlechner, Allison R. Najafi, Ming J. Lim, Ming J. Lim, David Smith, Frances M. Platt, Brenda P. Williams, Jonathan D. Cooper, Jonathan D. Cooper, Jonathan D. Cooper

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Regional and cellular neuropathology in the palmitoyl protein thioesterase-1 null mutant mouse model of infantile neuronal ceroid lipofuscinosis von Ellen Bible, Praveena Gupta, Sandra L Hofmann, Jonathan D Cooper

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Anti-inflammatory impact of minocycline in a mouse model of tauopathy von Claire J Garwood, Jonathan D Cooper, Diane P Hanger, Wendy Noble

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Loss of amyloid precursor protein in a mouse model of Niemann–Pick type C disease exacerbates its phenotype and disrupts tau homeostasis von Ana Nunes, Sarah N.R. Pressey, Jonathan D. Cooper, Salvador Soriano

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Selective spatiotemporal patterns of glial activation and neuron loss in the sensory thalamocortical pathways of neuronal ceroid lipofuscinosis 8 mice von Mervi Kuronen, Anna-Elina Lehesjoki, Anu Jalanko, Jonathan D. Cooper, Outi Kopra

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Thalamocortical neuron loss and localized astrocytosis in the Cln3Δex7/8 knock-in mouse model of Batten disease von Charlie C. Pontikis, Susan L. Cotman, Marcy E. MacDonald, Jonathan D. Cooper

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Neurodegeneration and Epilepsy in a Zebrafish Model of CLN3 Disease (Batten Disease). von Kim Wager, Anselm A Zdebik, Sonia Fu, Jonathan D Cooper, Robert J Harvey, Claire Russell

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Early glial activation, synaptic changes and axonal pathology in the thalamocortical system of Niemann–Pick type C1 mice von Sarah N.R. Pressey, David A. Smith, Andrew M.S. Wong, Frances M. Platt, Jonathan D. Cooper

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

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A murine model of variant late infantile ceroid lipofuscinosis recapitulates behavioral and pathological phenotypes of human disease. von Jeremy P Morgan, Helen Magee, Andrew Wong, Tarah Nelson, Bettina Koch, Jonathan D Cooper, Jill M Weimer

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Disruption of the autophagy-lysosome pathway is involved in neuropathology of the nclf mouse model of neuronal ceroid lipofuscinosis. von Melanie Thelen, Markus Damme, Michaela Schweizer, Christian Hagel, Andrew M S Wong, Jonathan D Cooper, Thomas Braulke, Giovanna Galliciotti

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Glial activation spreads from specific cerebral foci and precedes neurodegeneration in presymptomatic ovine neuronal ceroid lipofuscinosis (CLN6) von Manfred J. Oswald, David N. Palmer, Graham W. Kay, Stephen J.A. Shemilt, Payam Rezaie, Jonathan D. Cooper

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Compromised astrocyte function and survival negatively impact neurons in infantile neuronal ceroid lipofuscinosis von Jenny Lange, Luke J. Haslett, Emyr Lloyd-Evans, Jennifer M. Pocock, Mark S. Sands, Brenda P. Williams, Jonathan D. Cooper

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Genetically Corrected iPSC-Derived Neural Stem Cell Grafts Deliver Enzyme Replacement to Affect CNS Disease in Sanfilippo B Mice von Don Clarke, Yewande Pearse, Shih-hsin Kan, Steven Q. Le, Valentina Sanghez, Jonathan D. Cooper, Patricia I. Dickson, Michelina Iacovino

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Exacerbated neuronal ceroid lipofuscinosis phenotype in Cln1/5 double-knockout mice von Tea Blom, Mia-Lisa Schmiedt, Andrew M. Wong, Aija Kyttälä, Jarkko Soronen, Matti Jauhiainen, Jaana Tyynelä, Jonathan D. Cooper, Anu Jalanko

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Progressive thalamocortical neuron loss in Cln5 deficient mice: Distinct effects in Finnish variant late infantile NCL von Carina von Schantz, Catherine Kielar, Stine N. Hansen, Charlie C. Pontikis, Noreen A. Alexander, Outi Kopra, Anu Jalanko, Jonathan D. Cooper

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

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Visual deficits in a mouse model of Batten disease are the result of optic nerve degeneration and loss of dorsal lateral geniculate thalamic neurons von Jill M. Weimer, Andrew W. Custer, Jared W. Benedict, Noreen A. Alexander, Evan Kingsley, Howard J. Federoff, Jonathan D. Cooper, David A. Pearce

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