about
A novel somatodendritic marker defined by a peptide derived from the ALS2 proteinA murine model of variant late infantile ceroid lipofuscinosis recapitulates behavioral and pathological phenotypes of human diseaseNeurodegeneration and Epilepsy in a Zebrafish Model of CLN3 Disease (Batten Disease)FGFR3 regulates brain size by controlling progenitor cell proliferation and apoptosis during embryonic developmentInactivation of bcl-2 results in progressive degeneration of motoneurons, sympathetic and sensory neurons during early postnatal developmentVisual deficits in a mouse model of Batten disease are the result of optic nerve degeneration and loss of dorsal lateral geniculate thalamic neuronsCathepsin F mutations cause Type B Kufs disease, an adult-onset neuronal ceroid lipofuscinosisDHHC5 interacts with PDZ domain 3 of post-synaptic density-95 (PSD-95) protein and plays a role in learning and memory.Overexpression of human wild-type FUS causes progressive motor neuron degeneration in an age- and dose-dependent fashion.Cerebellar defects in a mouse model of juvenile neuronal ceroid lipofuscinosis.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.mTORC1-independent TFEB activation via Akt inhibition promotes cellular clearance in neurodegenerative storage diseasesBrain gene expression profiles of Cln1 and Cln5 deficient mice unravels common molecular pathways underlying neuronal degeneration in NCL diseases.Evolution of structural abnormalities in the rat brain following in utero exposure to maternal immune activation: A longitudinal in vivo MRI study.Cross-region reduction in 5-hydroxymethylcytosine in Alzheimer's disease brain.A metabolomic comparison of mouse models of the Neuronal Ceroid Lipofuscinoses.Immunosuppression alters disease severity in juvenile Batten disease miceAn anti-neuroinflammatory that targets dysregulated glia enhances the efficacy of CNS-directed gene therapy in murine infantile neuronal ceroid lipofuscinosisNeurotrophic factors as potential therapeutic agents in neuronal ceroid lipofuscinosis.Disruption of the autophagy-lysosome pathway is involved in neuropathology of the nclf mouse model of neuronal ceroid lipofuscinosisNeuropathology in mouse models of mucopolysaccharidosis type I, IIIA and IIIBAge-dependent therapeutic effect of memantine in a mouse model of juvenile Batten diseasePathogenesis and therapies for infantile neuronal ceroid lipofuscinosis (infantile CLN1 disease).Insulin receptor substrate 2 is a negative regulator of memory formation.Temporary inhibition of AMPA receptors induces a prolonged improvement of motor performance in a mouse model of juvenile Batten diseaseA novel, long-lived, and highly engraftable immunodeficient mouse model of mucopolysaccharidosis type I.Effects of cis-regulatory variation differ across regions of the adult human brain.Synergistic effects of central nervous system-directed gene therapy and bone marrow transplantation in the murine model of infantile neuronal ceroid lipofuscinosisSystemic gene delivery following intravenous administration of AAV9 to fetal and neonatal mice and late-gestation nonhuman primates.Non-invasive molecular imaging of inflammatory macrophages in allograft rejectionProgress towards understanding disease mechanisms in small vertebrate models of neuronal ceroid lipofuscinosis.Alterations in striatal dopamine catabolism precede loss of substantia nigra neurons in a mouse model of juvenile neuronal ceroid lipofuscinosisExacerbated neuronal ceroid lipofuscinosis phenotype in Cln1/5 double-knockout mice.Early glial activation, synaptic changes and axonal pathology in the thalamocortical system of Niemann-Pick type C1 mice.Immune system irregularities in lysosomal storage disorders.Location and connectivity determine GABAergic interneuron survival in the brains of South Hampshire sheep with CLN6 neuronal ceroid lipofuscinosisCerebellar pathology and motor deficits in the palmitoyl protein thioesterase 1-deficient mouseProgressive thalamocortical neuron loss in Cln5 deficient mice: Distinct effects in Finnish variant late infantile NCL.Molecular correlates of axonal and synaptic pathology in mouse models of Batten disease.Efficacy of phosphodiesterase-4 inhibitors in juvenile Batten disease (CLN3).
P50
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P50
description
hulumtues
@sq
onderzoeker
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researcher
@en
հետազոտող
@hy
name
Jonathan D Cooper
@nl
Jonathan D Cooper
@sl
Jonathan D. Cooper
@en
Jonathan D. Cooper
@es
type
label
Jonathan D Cooper
@nl
Jonathan D Cooper
@sl
Jonathan D. Cooper
@en
Jonathan D. Cooper
@es
altLabel
Jon
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prefLabel
Jonathan D Cooper
@nl
Jonathan D Cooper
@sl
Jonathan D. Cooper
@en
Jonathan D. Cooper
@es
P1053
C-8117-2009
P106
P21
P31
P3829
P496
0000-0003-1339-4750