Combined Anti-inflammatory and Neuroprotective Treatments Have the Potential to Impact Disease Phenotypes in Cln3−/− Mice

by 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
Published 2019-09-01

Regional and cellular neuropathology in the palmitoyl protein thioesterase-1 null mutant mouse model of infantile neuronal ceroid lipofuscinosis

by Ellen Bible, Praveena Gupta, Sandra L Hofmann, Jonathan D Cooper
Published 2004-07-01

Anti-inflammatory impact of minocycline in a mouse model of tauopathy

by Claire J Garwood, Jonathan D Cooper, Diane P Hanger, Wendy Noble
Published 2010-10-01

Loss of amyloid precursor protein in a mouse model of Niemann–Pick type C disease exacerbates its phenotype and disrupts tau homeostasis

by Ana Nunes, Sarah N.R. Pressey, Jonathan D. Cooper, Salvador Soriano
Published 2011-06-01

Selective spatiotemporal patterns of glial activation and neuron loss in the sensory thalamocortical pathways of neuronal ceroid lipofuscinosis 8 mice

by Mervi Kuronen, Anna-Elina Lehesjoki, Anu Jalanko, Jonathan D. Cooper, Outi Kopra
Published 2012-09-01

Thalamocortical neuron loss and localized astrocytosis in the Cln3Δex7/8 knock-in mouse model of Batten disease

by Charlie C. Pontikis, Susan L. Cotman, Marcy E. MacDonald, Jonathan D. Cooper
Published 2005-12-01

Neurodegeneration and Epilepsy in a Zebrafish Model of CLN3 Disease (Batten Disease).

by Kim Wager, Anselm A Zdebik, Sonia Fu, Jonathan D Cooper, Robert J Harvey, Claire Russell
Published 2016-01-01

Location and connectivity determine GABAergic interneuron survival in the brains of South Hampshire sheep with CLN6 neuronal ceroid lipofuscinosis

by Manfred J. Oswald, David N. Palmer, Graham W. Kay, Karen J. Barwell, Jonathan D. Cooper
Published 2008-10-01

Early glial activation, synaptic changes and axonal pathology in the thalamocortical system of Niemann–Pick type C1 mice

by Sarah N.R. Pressey, David A. Smith, Andrew M.S. Wong, Frances M. Platt, Jonathan D. Cooper
Published 2012-03-01

A murine model of variant late infantile ceroid lipofuscinosis recapitulates behavioral and pathological phenotypes of human disease.

by Jeremy P Morgan, Helen Magee, Andrew Wong, Tarah Nelson, Bettina Koch, Jonathan D Cooper, Jill M Weimer
Published 2013-01-01

Correction: Disruption of the Autophagy-Lysosome Pathway Is Involved in Neuropathology of the nclf Mouse Model of Neuronal Ceroid Lipofuscinosis

by Melanie Thelen, Markus Damme, Michaela Schweizer, Christian Hagel, Andrew M.S. Wong, Jonathan D. Cooper, Thomas Braulke, Giovanna Galliciotti
Published 2012-01-01

Correction: Disruption of the Autophagy-Lysosome Pathway Is Involved in Neuropathology of the Mouse Model of Neuronal Ceroid Lipofuscinosis.

by Melanie Thelen, Markus Damme, Michaela Schweizer, Christian Hagel, Andrew M.S. Wong, Jonathan D. Cooper, Thomas Braulke, Giovanna Galliciotti
Published 2012-01-01

Disruption of the autophagy-lysosome pathway is involved in neuropathology of the nclf mouse model of neuronal ceroid lipofuscinosis.

by Melanie Thelen, Markus Damme, Michaela Schweizer, Christian Hagel, Andrew M S Wong, Jonathan D Cooper, Thomas Braulke, Giovanna Galliciotti
Published 2012-01-01

Glial activation spreads from specific cerebral foci and precedes neurodegeneration in presymptomatic ovine neuronal ceroid lipofuscinosis (CLN6)

by Manfred J. Oswald, David N. Palmer, Graham W. Kay, Stephen J.A. Shemilt, Payam Rezaie, Jonathan D. Cooper
Published 2005-10-01

Compromised astrocyte function and survival negatively impact neurons in infantile neuronal ceroid lipofuscinosis

by Jenny Lange, Luke J. Haslett, Emyr Lloyd-Evans, Jennifer M. Pocock, Mark S. Sands, Brenda P. Williams, Jonathan D. Cooper
Published 2018-08-01

Genetically Corrected iPSC-Derived Neural Stem Cell Grafts Deliver Enzyme Replacement to Affect CNS Disease in Sanfilippo B Mice

by Don Clarke, Yewande Pearse, Shih-hsin Kan, Steven Q. Le, Valentina Sanghez, Jonathan D. Cooper, Patricia I. Dickson, Michelina Iacovino
Published 2018-09-01

Exacerbated neuronal ceroid lipofuscinosis phenotype in Cln1/5 double-knockout mice

by Tea Blom, Mia-Lisa Schmiedt, Andrew M. Wong, Aija Kyttälä, Jarkko Soronen, Matti Jauhiainen, Jaana Tyynelä, Jonathan D. Cooper, Anu Jalanko
Published 2013-03-01

Progressive thalamocortical neuron loss in Cln5 deficient mice: Distinct effects in Finnish variant late infantile NCL

by Carina von Schantz, Catherine Kielar, Stine N. Hansen, Charlie C. Pontikis, Noreen A. Alexander, Outi Kopra, Anu Jalanko, Jonathan D. Cooper
Published 2009-05-01

Successive neuron loss in the thalamus and cortex in a mouse model of infantile neuronal ceroid lipofuscinosis

by Catherine Kielar, Lucy Maddox, Ellen Bible, Charlie C. Pontikis, Shannon L. Macauley, Megan A. Griffey, Michael Wong, Mark S. Sands, Jonathan D. Cooper
Published 2007-01-01

IgG entry and deposition are components of the neuroimmune response in Batten disease

by Ming J. Lim, Noreen Alexander, Jared W. Benedict, Subrata Chattopadhyay, Stephen J.A. Shemilt, Christopher J. Guérin, Jonathan D. Cooper, David A. Pearce
Published 2007-02-01