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

Få fuldtekst

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

Få fuldtekst

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

Få fuldtekst

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

Få fuldtekst

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

Få fuldtekst

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

Få fuldtekst

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

Få fuldtekst

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

Få fuldtekst

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

Få fuldtekst

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

Få fuldtekst

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

Få fuldtekst

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

Få fuldtekst

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

Få fuldtekst

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

Få fuldtekst

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

Få fuldtekst

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

Få fuldtekst

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

Få fuldtekst

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

Få fuldtekst

Effects of chronic cannabidiol in a mouse model of naturally occurring neuroinflammation, neurodegeneration, and spontaneous seizures af 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

Få fuldtekst

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

Få fuldtekst