J. Cooper

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Laser Microdissection for Research into the MolecularNeuropathologyof BattenDisease

J. Cooper, 2006 .

CRISPR/Cas9 mediated generation of an ovine model for infantile neuronal ceroid lipofuscinosis (CLN1 disease)

C. Whitelaw, S. Lillico, J. Cooper, 2019, Scientific Reports.

Fetal gene therapy for neurodegenerative disease of infants

Kevin Mills, Arijit Biswas, Sebastian Brandner, 2018, Nature Medicine.

A zebrafish model of CLN2 disease is deficient in tripeptidyl peptidase 1 and displays progressive neurodegeneration accompanied by a reduction in proliferation.

Stephen W. Wilson, J. Cooper, C. Russell, 2013, Brain : a journal of neurology.

Overexpression of human wild-type FUS causes progressive motor neuron degeneration in an age- and dose-dependent fashion

T. Hortobágyi, C. Shaw, Bradley N Smith, 2012, Acta Neuropathologica.

Diagnosis of neuronal ceroid lipofuscinosis type 2 (CLN2 disease): Expert recommendations for early detection and laboratory diagnosis.

J. Cooper, R. Giugliani, Z. Lukacs, 2016, Molecular genetics and metabolism.

Laser Cordectomy for T1 Glottic Carcinoma: A 10-Year Experience and Videostroboscopic Findings

J. R. Chandler, J. Cooper, R. Casiano, 1991, Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery.

Insulin receptor substrate 2 is a negative regulator of memory formation.

S. Krauss, Mark A. Smith, J. Cooper, 2011, Learning & memory.

Expression of the Fe65 adapter protein in adult and developing mouse brain

C. Shaw, J. Cooper, K. Lau, 2002, Neuroscience.

Loss of neuronal cell cycle control as a mechanism of neurodegeneration in the Presenilin-1 Alzheimer's disease brain

T. Hortobágyi, B. Malik, J. Cooper, 2008, Cell cycle.

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

J. Cooper, M. Sands, E. Lloyd-Evans, 2018, Acta Neuropathologica Communications.

Neuropathology in Mouse Models of Mucopolysaccharidosis Type I, IIIA and IIIB

J. Cooper, S. Jones, E. Kremer, 2012, PloS one.

Synergistic effects of central nervous system‐directed gene therapy and bone marrow transplantation in the murine model of infantile neuronal ceroid lipofuscinosis

J. Cooper, M. Sands, A. Wong, 2012, Annals of neurology.

Visual deficits in a mouse model of Batten disease are the result of optic nerve degeneration and loss of dorsal lateral geniculate thalamic neurons

J. Cooper, H. Federoff, Evan P. Kingsley, 2006, Neurobiology of Disease.

A Murine Model of Variant Late Infantile Ceroid Lipofuscinosis Recapitulates Behavioral and Pathological Phenotypes of Human Disease

J. Cooper, J. Weimer, H. Magee, 2013, PloS one.

Intravenous high-dose enzyme replacement therapy with recombinant palmitoyl-protein thioesterase reduces visceral lysosomal storage and modestly prolongs survival in a preclinical mouse model of infantile neuronal ceroid lipofuscinosis.

L. Hynan, J. Cooper, S. Birnbaum, 2012, Molecular genetics and metabolism.

NCL disease mechanisms.

J. Cooper, J. Tyynelä, D. Palmer, 2013, Biochimica et biophysica acta.

Activation of Non‐neuronal Cells within the Prenatal Developing Brain of Sheep with Neuronal Ceroid Lipofuscinosis

J. Cooper, P. Rezaie, G. W. Kay, 2006, Brain pathology.

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

J. Cooper, M. Oswald, G. W. Kay, 2005, Neurobiology of Disease.

Evolution of structural abnormalities in the rat brain following in utero exposure to maternal immune activation: A longitudinal in vivo MRI study

Steven C. R. Williams, W. Crum, S. Sawiak, 2017, Brain, Behavior, and Immunity.

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

J. Cooper, M. Jauhiainen, J. Soronen, 2012, Disease Models & Mechanisms.

Nonclinical evaluation of CNS-administered TPP1 enzyme replacement in canine CLN2 neuronal ceroid lipofuscinosis.

J. Cooper, Joshua Henshaw, C. O’Neill, 2015, Molecular genetics and metabolism.

In utero administration of Ad5 and AAV pseudotypes to the fetal brain leads to efficient, widespread and long-term gene expression

J. Cooper, A. Baker, A. Rahim, 2011, Gene Therapy.

Intravenous administration of AAV2/9 to the fetal and neonatal mouse leads to differential targeting of CNS cell types and extensive transduction of the nervous system

J. Cooper, Seng H. Cheng, J. Chan, 2011, FASEB journal : official publication of the Federation of American Societies for Experimental Biology.

An update on the progress of preclinical models for guiding therapeutic management of neuronal ceroid lipofuscinosis

J. Cooper, H. R. Nelvagal, Hemanth R. Nelvagal, 2019 .

Brain transplantation of genetically corrected Sanfilippo type B neural stem cells induces partial cross-correction of the disease

M. Iacovino, J. Cooper, P. Dickson, 2022, bioRxiv.

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

J. Cooper, M. Schweizer, C. Hagel, 2012, PloS one.

Circadian rhythm and suprachiasmatic nucleus alterations in the mouse model of mucopolysaccharidosis IIIB

J. Cooper, B. Bigger, F. Wilkinson, 2010, Behavioural Brain Research.

Progress towards understanding the neurobiology of Batten disease or neuronal ceroid lipofuscinosis

J. Cooper, 2003, Current opinion in neurology.

Comparative proteomic profiling reveals mechanisms for early spinal cord vulnerability in CLN1 disease

J. Cooper, D. Lamont, S. Eaton, 2020, Scientific Reports.

Immune system irregularities in lysosomal storage disorders

J. Cooper, D. Pearce, J. Castaneda, 2008, Acta Neuropathologica.

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

J. Cooper, S. Soriano, A. Nunes, 2011, Neurobiology of Disease.

Trehalose reduces retinal degeneration, neuroinflammation and storage burden caused by a lysosomal hydrolase deficiency

S. Wu, M. Passafaro, J. Cooper, 2018, Autophagy.

Cln5-deficiency in mice leads to microglial activation, defective myelination and changes in lipid metabolism

E. Ikonen, J. Cooper, M. Jauhiainen, 2012, Neurobiology of Disease.

Efficient gene delivery to the adult and fetal CNS using pseudotyped non-integrating lentiviral vectors

N. Philpott, J. Cooper, A. Rahim, 2009, Gene Therapy.

Glial Dysfunction and Its Contribution to the Pathogenesis of the Neuronal Ceroid Lipofuscinoses

J. Cooper, Jenny Lange, Keigo Takahashi, 2022, Frontiers in Neurology.

Cerebellar pathology and motor deficits in the palmitoyl protein thioesterase 1-deficient mouse

J. Cooper, D. Wozniak, M. Sands, 2009, Experimental Neurology.

Intraventricular enzyme replacement improves disease phenotypes in a mouse model of late infantile neuronal ceroid lipofuscinosis.

B. Davidson, J. Cooper, Seng H. Cheng, 2008, Molecular therapy : the journal of the American Society of Gene Therapy.

Strategies for data analyses in a high resolution 1H NMR based metabolomics study of a mouse model of Batten disease

R. Mortishire-Smith, J. Griffin, J. Cooper, 2007, Metabolomics.

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

J. Cooper, C. Guérin, D. Pearce, 2007, Neurobiology of Disease.

The neuronal adaptor protein X11alpha reduces Abeta levels in the brains of Alzheimer's APPswe Tg2576 transgenic mice.

J. Cooper, K. Lau, Ju-Hyun Lee, 2003, The Journal of biological chemistry.

Brain-derived neurotrophic factor and neurotrophin-4 increase neurotrophin-3 expression in the rat hippocampus

E. Castrén, H. Thoenen, J. Cooper, 1994, International Journal of Developmental Neuroscience.

Partial rescue of neuropathology in the murine model of PKU following administration of recombinant phenylalanine ammonia lyase (pegvaliase).

P. Laipis, W. Zeile, J. Cooper, 2017, Molecular genetics and metabolism.

Translating preclinical models of neuronal ceroid lipofuscinosis: progress and prospects

J. Cooper, H. R. Nelvagal, Hemanth R. Nelvagal, 2017 .

Brain gene expression profiles of Cln1 and Cln5 deficient mice unravels common molecular pathways underlying neuronal degeneration in NCL diseases

L. Peltonen, M. Gentile, J. Cooper, 2008, BMC Genomics.

Identification of a Novel, Membrane-Associated Neuronal Kinase, Cyclin-Dependent Kinase 5/p35-Regulated Kinase

C. Shaw, P. Leigh, P. Leigh, 2003, The Journal of Neuroscience.

Progress towards understanding the neurobiology of Batten disease or neuronal ceroid lipofuscinosis.

J. Cooper, 2003 .

Molecular correlates of axonal and synaptic pathology in mouse models of Batten disease

J. Cooper, T. Gillingwater, M. Sands, 2009, Human molecular genetics.

A novel somatodendritic marker defined by a peptide derived from the ALS2 protein

P. Leigh, S. Skaper, J. Cooper, 2004, Neuroreport.

Clinical challenges and future therapeutic approaches for neuronal ceroid lipofuscinosis

S. Berkovic, J. Cooper, D. Medina, 2019, The Lancet Neurology.

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

J. Cooper, M. Sands, T. Wishart, 2022, Scientific Reports.

Longitudinal in vivo maturational changes of metabolites in the prefrontal cortex of rats exposed to polyinosinic–polycytidylic acid in utero

S. Kapur, Steven C. R. Williams, D. Lythgoe, 2015, European Neuropsychopharmacology.

p38alpha stress-activated protein kinase phosphorylates neurofilaments and is associated with neurofilament pathology in amyotrophic lateral sclerosis.

C. Shaw, P. Leigh, M. Ward, 2004, Molecular and cellular neurosciences.

Ganglioside GM1 potentiates NGF action on axotomised medial septal cholinergic neurons

M. Sofroniew, J. Cooper, M. Panni, 1998, Brain Research.

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

J. Cooper, A. Jalanko, C. Kielar, 2009, Neurobiology of Disease.

Pathogenesis and Therapies for Infantile Neuronal Ceroid Lipofuscinosis (infantile Cln1 Disease) Hhs Public Access

J. Cooper, M. Sands, Jacqueline A. Hawkins-Salsbury, 2022 .

Modelling Neurological Diseases in Large Animals: Criteria for Model Selection and Clinical Assessment

C. Whitelaw, S. Lillico, J. Cooper, 2022, Cells.

Non-invasive molecular imaging of inflammatory macrophages in allograft rejection

J. Cooper, P. Crocker, K. Brown, 2015, EJNMMI Research.

A Humoral Immune Response Alters the Distribution of Enzyme Replacement Therapy in Murine Mucopolysaccharidosis Type I

M. Iacovino, J. Cooper, M. Sands, 2017, Molecular therapy. Methods & clinical development.

Deregulation of biometal homeostasis: the missing link for neuronal ceroid lipofuscinoses?

P. Crouch, J. Cooper, J. Koistinaho, 2014, Metallomics : integrated biometal science.

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

Claire Russell, Jonathan D Cooper, Kim Wager, 2016, PloS one.

Regional Brain Atrophy in Mouse Models of Neuronal Ceroid Lipofuscinosis

J. Cooper, A. Wong, S. Dihanich, 2013, Journal of child neurology.

Age-dependent therapeutic effect of memantine in a mouse model of juvenile Batten disease

Angelika Saje, J. Cooper, D. Pearce, 2012, Neuropharmacology.

Temporary inhibition of AMPA receptors induces a prolonged improvement of motor performance in a mouse model of juvenile Batten disease

Angelika Saje, J. Cooper, É. Szabó, 2011, Neuropharmacology.

Immunosuppression alters disease severity in juvenile Batten disease mice

J. Cooper, D. Pearce, Chun‐Hung Chan, 2011, Journal of Neuroimmunology.

In utero gene transfer to the mouse nervous system.

D. Peebles, J. Chan, A. David, 2010, Biochemical Society transactions.

Neurotrophic mechanisms and neuronal degeneration

M. Sofroniew, J. Cooper, 1993 .

CHAPTER 13 – Neurotrophic effects on afferent neurons in the developing, adult and aged cholinergic basal forebrain

M. Sofroniew, J. Cooper, 1993 .

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

J. Cooper, K. J. Barwell, M. Oswald, 2008, Neurobiology of Disease.

Cerebellar defects in a mouse model of juvenile neuronal ceroid lipofuscinosis

J. Cooper, D. Pearce, Charlie C. Pontikis, 2009, Brain Research.

Cholinergic regulation of brain-derived neurotrophic factor (BDNF) and nerve growth factor (NGF) but not neurotrophin-3 (NT-3) mRNA levels in the developing rat hippocampus

E. Castrén, H. Thoenen, M. Sofroniew, 1993, The Journal of neuroscience : the official journal of the Society for Neuroscience.

FGFR3 regulates brain size by controlling progenitor cell proliferation and apoptosis during embryonic development.

J. Cooper, F. Pellicanò, Charlie C. Pontikis, 2005, Developmental biology.

CNS-directed AAV2-mediated gene therapy ameliorates functional deficits in a murine model of infantile neuronal ceroid lipofuscinosis.

J. Cooper, D. Wozniak, M. Sands, 2006, Molecular therapy : the journal of the American Society of Gene Therapy.

Proteomic mapping of differentially vulnerable pre-synaptic populations identifies regulators of neuronal stability in vivo

J. Cooper, S. Eaton, T. Gillingwater, 2017, Scientific Reports.

Proteomic mapping of differentially vulnerable pre-synaptic populations identifies regulators of neuronal stability in vivo

J. Cooper, S. Eaton, T. Gillingwater, 2017, Scientific Reports.

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

J. Cooper, F. Platt, David A Smith, 2012, Neurobiology of Disease.

Intravenous administration of scAAV9-Hexb normalizes lifespan and prevents pathology in Sandhoff disease mice

J. Cooper, C. Caillaud, M. Vanier, 2018, Human molecular genetics.

Value of needle biopsy in directing management of parotid lesions in HIV‐positive patients

J. Cooper, R. Casiano, E. Gould, 1991, Head & neck.

Effects of cis-regulatory variation differ across regions of the adult human brain.

Federica Buonocore, Claire Troakes, Jonathan D Cooper, 2010, Human molecular genetics.

A metabolomic comparison of mouse models of the Neuronal Ceroid Lipofuscinoses

R. Salek, R. Mortishire-Smith, J. Griffin, 2011, Journal of biomolecular NMR.

Targeted Disruption of the Cln3 Gene Provides a Mouse Model for Batten Disease

Jonathan D. Cooper, Robert L. Nussbaum, Peter E.M. Taschner, 1999, Neurobiology of Disease.

Apparent Loss and Hypertrophy of Interneurons in a Mouse Model of Neuronal Ceroid Lipofuscinosis: Evidence for Partial Response to Insulin-Like Growth Factor-1 Treatment

W. Mobley, J. Cooper, A. Messer, 1999, The Journal of Neuroscience.

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

Jonathan D. Cooper, J. Cooper, A. Jalanko, 2012, Neurobiology of Disease.

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

J. Cooper, M. Sands, C. Kielar, 2007, Neurobiology of Disease.

Thalamocortical neuron loss and localized astrocytosis in the Cln3 Δex7/8 knock-in mouse model of Batten disease

M. MacDonald, J. Cooper, S. Cotman, 2005, Neurobiology of Disease.

Moving towards therapies for Juvenile Batten disease?

J. Cooper, 2008, Experimental Neurology.

Progress towards understanding disease mechanisms in small vertebrate models of neuronal ceroid lipofuscinosis.

J. Cooper, H. Mitchison, C. Russell, 2006, Biochimica et biophysica acta.

Late onset neurodegeneration in the Cln3 −/− mouse model of juvenile neuronal ceroid lipofuscinosis is preceded by low level glial activation

W. Mobley, J. Cooper, P. Rezaie, 2004, Brain Research.

Selectivity and Types of Cell Death in the Neuronal Ceroid Lipofuscinoses (NCLs)

J. Cooper, H. Mitchison, Ming Lim, 2004, Brain pathology.

An autoantibody inhibitory to glutamic acid decarboxylase in the neurodegenerative disorder Batten disease.

Masumi Ito, Jonathan D Cooper, J. Cooper, 2002, Human molecular genetics.

9 Neurotrophic factors as potential therapeutic agents in neuronal ceroid lipofuscinosis

W. Mobley, J. Cooper, 2001 .

Top-down and bottom-up propagation of disease in the neuronal ceroid lipofuscinoses

J. Cooper, J. Ostergaard, H. R. Nelvagal, 2022, Frontiers in Neurology.

Neuroprotection of host cells by human central nervous system stem cells in a mouse model of infantile neuronal ceroid lipofuscinosis.

I. Weissman, F. Gage, W. Mobley, 2009, Cell stem cell.

mTORC1-independent TFEB activation via Akt inhibition promotes cellular clearance in neurodegenerative storage diseases

J. Neilson, S. Wu, R. Pautler, 2017, Nature Communications.

Towards a new understanding of NCL pathogenesis.

J. Cooper, Marta A. Tarczyluk, H. R. Nelvagal, 2015, Biochimica et biophysica acta.

Intracranial delivery of AAV9 gene therapy partially prevents retinal degeneration and visual deficits in CLN6-Batten disease mice

Tyler B. Johnson, J. Cooper, B. Kaspar, 2021, Molecular therapy. Methods & clinical development.

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

J. Cooper, F. Platt, A. Najafi, 2019, Front. Neurol..

Efficacy of phosphodiesterase‐4 inhibitors in juvenile Batten disease (CLN3)

J. Cooper, Megan E Bosch, A. Aldrich, 2016, Annals of neurology.

Partial correction of the CNS lysosomal storage defect in a mouse model of juvenile neuronal ceroid lipofuscinosis by neonatal CNS administration of an adeno-associated virus serotype rh.10 vector expressing the human CLN3 gene.

R. Crystal, N. Hackett, J. Cooper, 2014, Human gene therapy.

Galactolipid deficiency in the early pathogenesis of neuronal ceroid lipofuscinosis model Cln8mnd: implications to delayed myelination and oligodendrocyte maturation

J. Cooper, M. Hermansson, P. Somerharju, 2012, Neuropathology and applied neurobiology.

Distinct patterns of serum immunoreactivity as evidence for multiple brain‐directed autoantibodies in juvenile neuronal ceroid lipofuscinosis

J. Cooper, D. Pearce, E. Bible, 2006, Neuropathology and applied neurobiology.

Hippocampal Pathology in the Human Neuronal Ceroid‐Lipofuscinoses: Distinct Patterns of Storage Deposition, Neurodegeneration and Glial Activation

J. Cooper, M. Haltia, M. N. Khan, 2004, Brain pathology.

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

J. Cooper, E. Bible, S. Hofmann, 2004, Neurobiology of Disease.

Enhanced expression of manganese-dependent superoxide dismutase in human and sheep CLN6 tissues.

J. Cooper, T. Braulke, M. Elleder, 2003, The Biochemical journal.

Distinct Neuropathologic Phenotypes After Disrupting the Chloride Transport Proteins ClC-6 or ClC-7/Ostm1

J. Cooper, T. Jentsch, T. Stauber, 2010, Journal of neuropathology and experimental neurology.

Current therapies for the soluble lysosomal forms of neuronal ceroid lipofuscinosis.

J. Cooper, A. Rahim, S. Waddington, 2010, Biochemical Society transactions.

Immune cells perturb axons and impair neuronal survival in a mouse model of infantile neuronal ceroid lipofuscinosis.

T. Langmann, J. Cooper, R. Martini, 2013, Brain : a journal of neurology.

Neuronal Ceroid Lipofuscinosis

J. Cooper, 2010 .

Synaptic Changes in the Thalamocortical System of Cathepsin D-Deficient Mice: A Model of Human Congenital Neuronal Ceroid-Lipofuscinosis

P. Saftig, J. Cooper, T. Gillingwater, 2008, Journal of neuropathology and experimental neurology.

High Resolution 1H NMR-based Metabolomics Indicates a Neurotransmitter Cycling Deficit in Cerebral Tissue from a Mouse Model of Batten Disease*

Julian L Griffin, Jonathan D Cooper, R. Mortishire-Smith, 2005, Journal of Biological Chemistry.

Adeno-associated virus 2-mediated gene therapy decreases autofluorescent storage material and increases brain mass in a murine model of infantile neuronal ceroid lipofuscinosis

J. Cooper, M. Sands, E. Bible, 2004, Neurobiology of Disease.

Glial cells are functionally impaired in juvenile neuronal ceroid lipofuscinosis and detrimental to neurons

J. Cooper, S. Heales, P. Rezaie, 2017, Acta Neuropathologica Communications.

Spinal manifestations of CLN1 disease start during the early postnatal period

J. Cooper, M. Sands, J. Dearborn, 2020, Neuropathology and applied neurobiology.

Future perspectives: What lies ahead for Neuronal Ceroid Lipofuscinosis research?

J. Cooper, S. Mole, 2020, Biochimica et biophysica acta. Molecular basis of disease.

Pathomechanisms in the neuronal ceroid lipofuscinoses.

J. Cooper, Jenny Lange, Keigo Takahashi, 2020, Biochimica et biophysica acta. Molecular basis of disease.

CRISPR/Cas9 mediated generation of an ovine model for infantile neuronal ceroid lipofuscinosis (CLN1 disease)

C. Whitelaw, S. Lillico, J. Cooper, 2019, Scientific Reports.

Characterization of a novel porcine model of CLN3-Batten disease

Tyler B. Johnson, J. Cooper, A. Drack, 2019, Molecular Genetics and Metabolism.

Synergistic effects of treating the spinal cord and brain in CLN1 disease

R. Schmidt, J. Cooper, M. Sands, 2017, Proceedings of the National Academy of Sciences.

Early Microglial Activation Precedes Neuronal Loss in the Brain of the Cstb−/− Mouse Model of Progressive Myoclonus Epilepsy, EPM1

J. Cooper, T. Joensuu, A. Lehesjoki, 2012, Journal of neuropathology and experimental neurology.

An Anti-Neuroinflammatory That Targets Dysregulated Glia Enhances the Efficacy of CNS-Directed Gene Therapy in Murine Infantile Neuronal Ceroid Lipofuscinosis

J. Cooper, M. Sands, S. Fowler, 2014, The Journal of Neuroscience.

Combination small molecule PPT1 mimetic and CNS-directed gene therapy as a treatment for infantile neuronal ceroid lipofuscinosis

J. Cooper, M. Sands, A. Wong, 2012, Journal of Inherited Metabolic Disease.

Cross-species efficacy of enzyme replacement therapy for CLN1 disease in mice and sheep

S. Ekins, C. Whitelaw, S. Lillico, 2022, The Journal of clinical investigation.

Intrathecal enzyme replacement therapy improves motor function and survival in a preclinical mouse model of infantile neuronal ceroid lipofuscinosis.

J. Cooper, S. Birnbaum, S. Hofmann, 2015, Molecular genetics and metabolism.

Cortical interneuron loss and seizure generation as novel clinically relevant disease phenotypes in Cln2R207X mice

J. Cooper, M. Sands, N. Rensing, 2022, bioRxiv.

Inactivation of bcl-2 Results in Progressive Degeneration of Motoneurons, Sympathetic and Sensory Neurons during Early Postnatal Development

H. Thoenen, J. Cooper, B. Holtmann, 1996, Neuron.

Trafficking the NGF signal: implications for normal and degenerating neurons.

C. Howe, W. Mobley, J. Cooper, 2004, Progress in brain research.

Alterations in striatal dopamine catabolism precede loss of substantia nigra neurons in a mouse model of juvenile neuronal ceroid lipofuscinosis

Deborah A. Ryan, J. Cooper, H. Federoff, 2007, Brain Research.

An immunohistochemical and stereological analysis of PSI‐induced nigral neuronal degeneration in the rat

J. Cooper, P. Jenner, B. Zeng, 2009, Journal of neurochemistry.

Impairment of the ubiquitin-proteasome system is thought to contribute to the pathogenesis of Parkinson’s disease (PD) and to the formation of Lewy bodies

J. Cooper, P. Jenner, B. Zeng, 2009 .

A novel, long-lived, and highly engraftable immunodeficient mouse model of mucopolysaccharidosis type I

J. Jester, J. Cooper, E. Monuki, 2015, Molecular therapy. Methods & clinical development.

Absence of p75NTR Causes Increased Basal Forebrain Cholinergic Neuron Size, Choline Acetyltransferase Activity, and Target Innervation

L. Butcher, D. Bredesen, W. Mobley, 1997, The Journal of Neuroscience.

Reduced transport of [125I]nerve growth factor by cholinergic neurons and down-regulated trka expression in the medial septum of aged rats

M. Sofroniew, J. Cooper, D. Lindholm, 1994, Neuroscience.

Generation of light-producing somatic-transgenic mice using adeno-associated virus vectors

J. Cooper, E. Henckaerts, T. McKay, 2016, Scientific Reports.

Systemic gene delivery following intravenous administration of AAV9 to fetal and neonatal mice and late-gestation nonhuman primates

J. Chan, J. Cooper, A. Rahim, 2015, FASEB journal : official publication of the Federation of American Societies for Experimental Biology.

1 Cross-region reduction in 5-hydroxymethylcytosine in Alzheimer ' s disease brain

Katie, S. Lovestone, J. Mill, 2016 .

Cross-region reduction in 5-hydroxymethylcytosine in Alzheimer's disease brain

S. Lovestone, J. Mill, P. Proitsi, 2014, Neurobiology of Aging.

p38α stress-activated protein kinase phosphorylates neurofilaments and is associated with neurofilament pathology in amyotrophic lateral sclerosis

Jonathan D Cooper, C. Shaw, P. Leigh, 2004, Molecular and Cellular Neuroscience.

Recombinant NAGLU-IGF2 prevents physical and neurological disease and improves survival in Sanfilippo B syndrome

Jodi D. Smith, T. Chou, J. Cooper, 2021, bioRxiv.

Central nervous system pathology in preclinical MPS IIIB dogs reveals progressive changes in clinically relevant brain regions

J. Cooper, Emma McCullagh, N. M. Ellinwood, 2020, Scientific Reports.

Myelin and Lipid Composition of the Corpus Callosum in Mucopolysaccharidosis Type I Mice.

D. Elashoff, J. Tolar, J. Cooper, 2020, Lipids.

Biochemical evaluation of intracerebroventricular rhNAGLU-IGF2 enzyme replacement therapy in neonatal mice with Sanfilippo B syndrome.

J. Cooper, M. Sands, P. Dickson, 2021, Molecular genetics and metabolism.

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

M. Iacovino, J. Cooper, P. Dickson, 2018, Molecular therapy. Methods & clinical development.

Tralesinidase Alfa Enzyme Replacement Therapy Prevents Disease Manifestations in a Canine Model of Mucopolysaccharidosis Type IIIB

B. Mueller, Jodi D. Smith, J. Cooper, 2022, The Journal of Pharmacology and Experimental Therapeutics.

Reduced Cortical Volume and Elevated Astrocyte Density in Rats Chronically Treated With Antipsychotic Drugs—Linking Magnetic Resonance Imaging Findings to Cellular Pathology

Steven C.R. Williams, Jonathan D. Cooper, William R. Crum, 2014, Biological Psychiatry.

Spatial and temporal correlation between neuron loss and neuroinflammation in a mouse model of neuronopathic Gaucher disease.

J. Cooper, A. Futerman, R. Eilam, 2011, Human molecular genetics.

Morphologic and Functional Correlates of Synaptic Pathology in the Cathepsin D Knockout Mouse Model of Congenital Neuronal Ceroid Lipofuscinosis

A. Edwards, J. Cooper, A. Wright, 2011, Journal of neuropathology and experimental neurology.

Morphologic and Functional Correlates of Synaptic Pathology in the Cathepsin D Knockout Mouse Model of Congenital Neuronal Ceroid Lipofuscinosis

A. Edwards, J. Cooper, A. Wright, 2011, Journal of Neuropathology and Experimental Neurology.

Cathepsin F mutations cause Type B Kufs disease, an adult-onset neuronal ceroid lipofuscinosis.

Katherine R. Smith, F. Andermann, S. Berkovic, 2013, Human molecular genetics.

Delayed Death of Septal Cholinergic Neurons after Excitotoxic Ablation of Hippocampal Neurons during Early Postnatal Development in the Rat

M. Sofroniew, J. Skepper, J. Cooper, 1996, Experimental Neurology.

Anti-Inflammatory Impact of Minocycline in a Mouse Model of Tauopathy

J. Cooper, W. Noble, D. Hanger, 2010, Front. Psychiatry.

Reduced retrograde labelling with fluorescent tracer accompanies neuronal atrophy of basal forebrain cholinergic neurons in aged rats

M. Sofroniew, S. Lacalle, S. Dunnett, 1996, Neuroscience.

The RGD‐containing peptide fragment of osteopontin protects tyrosine hydroxylase positive cells against toxic insult in primary ventral mesencephalic cultures and in the rat substantia nigra

J. Cooper, P. Jenner, L. Broom, 2010, Journal of neurochemistry.

Contrasting Effects of Haloperidol and Lithium on Rodent Brain Structure: A Magnetic Resonance Imaging Study with Postmortem Confirmation

S. Kapur, Steven C. R. Williams, W. Crum, 2012, Biological Psychiatry.

DHHC5 Interacts with PDZ Domain 3 of Post-synaptic Density-95 (PSD-95) Protein and Plays a Role in Learning and Memory*

R. Hammer, J. Cooper, S. Birnbaum, 2010, The Journal of Biological Chemistry.

Microglial activation in the rat brain following chronic antipsychotic treatment at clinically relevant doses

S. Kapur, Steven C. R. Williams, S. Natesan, 2015, European Neuropsychopharmacology.

Trophic Factor Effects on Septal Cholinergic Neurons a

M. Sofroniew, C. Svendsen, J. Cooper, 1991, Annals of the New York Academy of Sciences.

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

J. Cooper, M. Schweizer, M. Damme, 2012, PLoS ONE.

Severe Central Nervous System Demyelination in Sanfilippo Disease

A. Pshezhetsky, Sheng-Kwei Song, T. Kennedy, 2023, bioRxiv.

Behavioral deficits, early gliosis, dysmyelination and synaptic dysfunction in a mouse model of mucolipidosis IV

R. J. Kelleher, B. Hyman, B. Bacskai, 2014, Acta neuropathologica communications.

Failed retrograde transport of NGF in a mouse model of Down's syndrome: Reversal of cholinergic neurodegenerative phenotypes following NGF infusion

Ahmad Salehi, Jonathan D. Cooper, Charles J. Epstein, 2001, Proceedings of the National Academy of Sciences of the United States of America.

Corrigendum: mTORC1-independent TFEB activation via Akt inhibition promotes cellular clearance in neurodegenerative storage diseases

J. Neilson, S. Wu, R. Pautler, 2017, Nature Communications.

The neuronal ceroid lipofuscinoses: the same, but different?

J. Cooper, 2010, Biochemical Society transactions.

The Neuronal Adaptor Protein X11α Reduces Aβ Levels in the Brains of Alzheimer's APPswe Tg2576 Transgenic Mice*

J. Cooper, K. Lau, Ju-Hyun Lee, 2003, Journal of Biological Chemistry.

A mouse mutant deficient in both neuronal ceroid lipofuscinosis‐associated proteins CLN3 and TPP1

J. Cooper, D. Sleat, P. Lobel, 2023, Journal of inherited metabolic disease.

Quantifying storage material accumulation in tissue sections.

J. Cooper, Hemanth R. Nelvagal, Helen R. Brooks, 2015, Methods in cell biology.

Increased vulnerability of septal cholinergic neurons to partial loss of target neurons in aged rats

M. Sofroniew, J. Cooper, 1996, Neuroscience.

Reply to: Lithium and the Expanding Brain

S. Kapur, Steven C. R. Williams, W. Crum, 2012, Biological Psychiatry.

Title: Bowel dysmotility and enteric neuron degeneration in lysosomal storage disease mice is prevented by gene therapy Short title: Treatment of enteric disease by gene therapy

J. Cooper, R. Heuckeroth, M. Sardiello, 2023 .

Gene therapy ameliorates spontaneous seizures associated with cortical neuron loss in a Cln2R207X mouse model

O. Danos, J. Cooper, M. Sands, 2023, The Journal of clinical investigation.

CRISPR/Cas9 mediated generation of an ovine model for infantile neuronal ceroid lipofuscinosis (CLN1 disease)

C. Whitelaw, S. Lillico, J. Cooper, 2019, Scientific Reports.

Investigative and emerging treatments for Batten disease

J. Cooper, Marta A Tarczyluk, 2015 .

Edinburgh Research Explorer Morphological and Functional Correlates of Synaptic Pathology in the Cathepsin D Knock-out Mouse Model of Congenital Neuronal Ceroid-lipofuscinosis Europe Pmc Funders Group

J. Cooper, A. Wright, T. Gillingwater, 2022 .

Guidelines for incorporating scientific knowledge and practice on rare diseases into higher education: neuronal ceroid lipofuscinoses as a model disorder.

J. Cooper, F. Platt, Ana María Oller de Ramírez, 2015, Biochimica et biophysica acta.

NMDA potentiates NGF-induced sprouting of septal cholinergic fibres.

J. Berke, M. Sofroniew, C. Heisenberg, 1994, Neuroreport.

NCL disease mechanisms.

J. Cooper, D. Palmer, Lucy A. Barry, 2013, Biochimica et biophysica acta.

Cln5-deficiency in mice leads to microglial activation, defective myelination and changes in lipid metabolism

E. Ikonen, J. Cooper, M. Jauhiainen, 2012, Neurobiology of Disease.

Pathogenesis and therapies for infantile neuronal ceroid lipofuscinosis (infantile CLN1 disease).

J. Cooper, M. Sands, Jacqueline A. Hawkins-Salsbury, 2013, Biochimica et Biophysica Acta.

Progress toward Fulfilling the Potential of Immunomodulation in Childhood Neurodegeneration?

J. Cooper, H. R. Nelvagal, Hemanth R. Nelvagal, 2017, Molecular therapy : the journal of the American Society of Gene Therapy.

Synergistic effects of treating the spinal cord and brain in CLN1 disease

R. Schmidt, J. Cooper, M. Sands, 2017, Proceedings of the National Academy of Sciences.

Synergistic effects of central nervous system‐directed gene therapy and bone marrow transplantation in the murine model of infantile neuronal ceroid lipofuscinosis

J. Cooper, M. Sands, A. Wong, 2012, Annals of neurology.

Immunosuppression alters disease severity in juvenile Batten disease mice

J. Cooper, D. Pearce, Chun‐Hung Chan, 2011, Journal of Neuroimmunology.

Efficient gene delivery to the adult and fetal CNS using pseudotyped non-integrating lentiviral vectors

N. Philpott, J. Cooper, A. Rahim, 2009, Gene Therapy.

Identification of a Novel, Membrane-Associated Neuronal Kinase, Cyclin-Dependent Kinase 5/p35-Regulated Kinase

C. Shaw, P. Leigh, J. Cooper, 2003, The Journal of Neuroscience.

Guidelines for incorporating scientific knowledge and practice on rare diseases into higher education: neuronal ceroid lipofuscinoses as a model disorder.

J. Cooper, F. Platt, Ana María Oller de Ramírez, 2015, Biochimica et biophysica acta.