Successive neuron loss in the thalamus and cortex in a mouse model of infantile neuronal ceroid lipofuscinosis.
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Moving towards effective therapeutic strategies for Neuronal Ceroid LipofuscinosisThe novel Cln1(R151X) mouse model of infantile neuronal ceroid lipofuscinosis (INCL) for testing nonsense suppression therapyA murine model of variant late infantile ceroid lipofuscinosis recapitulates behavioral and pathological phenotypes of human diseaseComprehensive functional characterization of murine infantile Batten disease including Parkinson-like behavior and dopaminergic markers.Uncovering molecular biomarkers that correlate cognitive decline with the changes of hippocampus' gene expression profiles in Alzheimer's disease.Rapamycin prevents epilepsy in a mouse model of tuberous sclerosis complex.Gene expression profiling in a mouse model of infantile neuronal ceroid lipofuscinosis reveals upregulation of immediate early genes and mediators of the inflammatory responseBrain gene expression profiles of Cln1 and Cln5 deficient mice unravels common molecular pathways underlying neuronal degeneration in NCL diseases.Treatment of the Ppt1(-/-) mouse model of infantile neuronal ceroid lipofuscinosis with the N-methyl-D-aspartate (NMDA) receptor antagonist memantine.A tailored mouse model of CLN2 disease: A nonsense mutant for testing personalized therapies.Combination small molecule PPT1 mimetic and CNS-directed gene therapy as a treatment for infantile neuronal ceroid lipofuscinosis.Possible involvement of lysosomal dysfunction in pathological changes of the brain in aged progranulin-deficient miceAn anti-neuroinflammatory that targets dysregulated glia enhances the efficacy of CNS-directed gene therapy in murine infantile neuronal ceroid lipofuscinosisBehavioral deficits, early gliosis, dysmyelination and synaptic dysfunction in a mouse model of mucolipidosis IV.Disruption of the autophagy-lysosome pathway is involved in neuropathology of the nclf mouse model of neuronal ceroid lipofuscinosisAge-dependent therapeutic effect of memantine in a mouse model of juvenile Batten diseaseEDITORIAL Neuroglia as a Central Element of Neurological Diseases: An Underappreciated Target for Therapeutic InterventionThe promise of an interneuron-based cell therapy for epilepsyGene expression alterations in the cerebellum and granule neurons of Cstb(-/-) mouse are associated with early synaptic changes and inflammation.Temporary inhibition of AMPA receptors induces a prolonged improvement of motor performance in a mouse model of juvenile Batten diseaseThe role of attenuated astrocyte activation in infantile neuronal ceroid lipofuscinosis.A novel, long-lived, and highly engraftable immunodeficient mouse model of mucopolysaccharidosis type I.Proteomic Profiling in the Brain of CLN1 Disease Model Reveals Affected Functional Modules.Synergistic effects of central nervous system-directed gene therapy and bone marrow transplantation in the murine model of infantile neuronal ceroid lipofuscinosisEvaluation of neurodegeneration in a mouse model of infantile batten disease by magnetic resonance imaging and magnetic resonance spectroscopy.Native and Complexed IGF-1: Biodistribution and Pharmacokinetics in Infantile Neuronal Ceroid Lipofuscinosis.Neuroinflammatory paradigms in lysosomal storage diseases.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.Suppression of agrin-22 production and synaptic dysfunction in Cln1 (-/-) mice.Histochemical localization of palmitoyl protein thioesterase-1 activityAlterations 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.Therapeutic approaches to the challenge of neuronal ceroid lipofuscinoses.Early glial activation, synaptic changes and axonal pathology in the thalamocortical system of Niemann-Pick type C1 mice.Ablation of neurons expressing agouti-related protein activates fos and gliosis in postsynaptic target regions.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.Neuropeptide changes and neuroactive amino acids in CSF from humans and sheep with neuronal ceroid lipofuscinoses (NCLs, Batten disease).
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P2860
Successive neuron loss in the thalamus and cortex in a mouse model of infantile neuronal ceroid lipofuscinosis.
description
2006 nî lūn-bûn
@nan
2006年の論文
@ja
2006年論文
@yue
2006年論文
@zh-hant
2006年論文
@zh-hk
2006年論文
@zh-mo
2006年論文
@zh-tw
2006年论文
@wuu
2006年论文
@zh
2006年论文
@zh-cn
name
Successive neuron loss in the ...... euronal ceroid lipofuscinosis.
@ast
Successive neuron loss in the ...... euronal ceroid lipofuscinosis.
@en
type
label
Successive neuron loss in the ...... euronal ceroid lipofuscinosis.
@ast
Successive neuron loss in the ...... euronal ceroid lipofuscinosis.
@en
prefLabel
Successive neuron loss in the ...... euronal ceroid lipofuscinosis.
@ast
Successive neuron loss in the ...... euronal ceroid lipofuscinosis.
@en
P2093
P2860
P1476
Successive neuron loss in the ...... euronal ceroid lipofuscinosis.
@en
P2093
Catherine Kielar
Charlie C Pontikis
Ellen Bible
Jonathan D Cooper
Lucy Maddox
Mark S Sands
Megan A Griffey
Michael Wong
Shannon L Macauley
P2860
P304
P356
10.1016/J.NBD.2006.09.001
P577
2006-10-12T00:00:00Z