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Effects of chronic cannabidiol in a mouse model of naturally occurring neuroinflammation, neurodegeneration, and spontaneous seizures.
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- Scientific Reports, 2022, v. 12, n. 1, p. 1, doi. 10.1038/s41598-022-15134-5
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- Article
Combined Anti-inflammatory and Neuroprotective Treatments Have the Potential to Impact Disease Phenotypes in Cln3 <sup>−/−</sup> Mice.
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- Frontiers in Neurology, 2019, p. 1, doi. 10.3389/fneur.2019.00963
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- Article
Identification of a Novel, Membrane-Associated Neuronal Kinase, Cyclin-Dependent Kinase 5/p35-Regulated Kinase.
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- Journal of Neuroscience, 2003, v. 23, n. 12, p. 4975, doi. 10.1523/JNEUROSCI.23-12-04975.2003
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- Publication type:
- Article
Value of needle biopsy in directing management of parotid lesions in HIV-positive patients.
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- Head & Neck, 1991, v. 13, n. 5, p. 411, doi. 10.1002/hed.2880130506
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- Publication type:
- Article
Glial Dysfunction and Its Contribution to the Pathogenesis of the Neuronal Ceroid Lipofuscinoses.
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- Frontiers in Neurology, 2022, v. 13, p. 1, doi. 10.3389/fneur.2022.886567
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- Publication type:
- Article
A Murine Model of Variant Late Infantile Ceroid Lipofuscinosis Recapitulates Behavioral and Pathological Phenotypes of Human Disease.
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- PLoS ONE, 2013, v. 8, n. 11, p. 1, doi. 10.1371/journal.pone.0078694
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- Publication type:
- Article
Neuropathology in Mouse Models of Mucopolysaccharidosis Type I, IIIA and IIIB.
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- PLoS ONE, 2012, v. 7, n. 4, p. 1, doi. 10.1371/journal.pone.0035787
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- Article
Disruption of the Autophagy-Lysosome Pathway Is Involved in Neuropathology of the nclf Mouse Model of Neuronal Ceroid Lipofuscinosis.
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- PLoS ONE, 2012, v. 7, n. 4, p. 1, doi. 10.1371/journal.pone.0035493
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- Publication type:
- Article
Compromised astrocyte function and survival negatively impact neurons in infantile neuronal ceroid lipofuscinosis.
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- Acta Neuropathologica Communications, 2018, v. 6, n. 1, p. N.PAG, doi. 10.1186/s40478-018-0575-4
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- Publication type:
- Article
Glial cells are functionally impaired in juvenile neuronal ceroid lipofuscinosis and detrimental to neurons.
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- Acta Neuropathologica Communications, 2017, v. 5, p. 1, doi. 10.1186/s40478-017-0476-y
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- Publication type:
- Article
Brain gene expression profiles of Cln1 and Cln5 deficient mice unravels common molecular pathways underlying neuronal degeneration in NCL diseases.
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- BMC Genomics, 2008, v. 9, p. 1, doi. 10.1186/1471-2164-9-146
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- Publication type:
- Article
Generation of light-producing somatic-transgenic mice using adeno-associated virus vectors.
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- Scientific Reports, 2020, v. 10, n. 1, p. 1, doi. 10.1038/s41598-020-59075-3
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- Publication type:
- Article
A zebrafish model of CLN2 disease is deficient in tripeptidyl peptidase 1 and displays progressive neurodegeneration accompanied by a reduction in proliferation.
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- Brain: A Journal of Neurology, 2013, v. 136, n. 5, p. 1488, doi. 10.1093/brain/awt043
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- Publication type:
- Article
Immune cells perturb axons and impair neuronal survival in a mouse model of infantile neuronal ceroid lipofuscinosis.
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- Brain: A Journal of Neurology, 2013, v. 136, n. 4, p. 1083, doi. 10.1093/brain/awt020
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- Publication type:
- Article
Systemic gene delivery following intravenous administration of AAV9 to fetal and neonatal mice and late-gestation nonhuman primates.
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- FASEB Journal, 2015, v. 29, n. 9, p. 3876, doi. 10.1096/fj.14-269092
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- Article
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.
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- FASEB Journal, 2011, v. 25, n. 10, p. 3505, doi. 10.1096/fj.11-182311
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- Article
Inflammation in the Lysosomal Storage Diseases.
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- Current Medical Literature: Lysosomal Storage Disease, 2012, v. 10, n. 3, p. 61
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- Article
Immune system irregularities in lysosomal storage disorders.
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- Acta Neuropathologica, 2008, v. 115, n. 2, p. 159, doi. 10.1007/s00401-007-0296-4
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- Publication type:
- Article
mTORC1-independent TFEB activation via Akt inhibition promotes cellular clearance in neurodegenerative storage diseases.
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- Nature Communications, 2017, v. 8, n. 2, p. 14338, doi. 10.1038/ncomms14338
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- Publication type:
- Article
Top-down and bottom-up propagation of disease in the neuronal ceroid lipofuscinoses.
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- Frontiers in Neurology, 2022, v. 13, p. 1, doi. 10.3389/fneur.2022.1061363
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- Publication type:
- Article
Activation of Non-neuronal Cells within the Prenatal Developing Brain of Sheep with Neuronal Ceroid Lipofuscinosis.
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- Brain Pathology, 2006, v. 16, n. 2, p. 110, doi. 10.1111/j.1750-3639.2006.00002.x
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- Publication type:
- Article
Hippocampal Pathology in the Human Neuronal Ceroid-Lipofuscinoses: Distinct Patterns of Storage Deposition, Neurodegeneration and Glial Activation.
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- Brain Pathology, 2004, v. 14, n. 4, p. 349, doi. 10.1111/j.1750-3639.2004.tb00077.x
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- Article
Selectivity and Types of Cell Death in the Neuronal Ceroid Lipofuscinoses (NCLs).
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- Brain Pathology, 2004, v. 14, n. 1, p. 86, doi. 10.1111/j.1750-3639.2004.tb00502.x
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- Article
Efficacy of phosphodiesterase-4 inhibitors in juvenile Batten disease (CLN3).
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- 2016
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- Publication type:
- journal article
Synergistic effects of central nervous system-directed gene therapy and bone marrow transplantation in the murine model of infantile neuronal ceroid lipofuscinosis.
- Published in:
- Annals of Neurology, 2012, v. 71, n. 6, p. 797, doi. 10.1002/ana.23545
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- Publication type:
- Article
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.
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- Journal of Neurochemistry, 2010, v. 114, n. 6, p. 1792, doi. 10.1111/j.1471-4159.2010.06896.x
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- Article
A mouse mutant deficient in both neuronal ceroid lipofuscinosis‐associated proteins CLN3 and TPP1.
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- Journal of Inherited Metabolic Disease, 2023, v. 46, n. 4, p. 720, doi. 10.1002/jimd.12619
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- Publication type:
- Article
Central nervous system pathology in preclinical MPS IIIB dogs reveals progressive changes in clinically relevant brain regions.
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- Scientific Reports, 2020, v. 10, n. 1, p. N.PAG, doi. 10.1038/s41598-020-77032-y
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- Publication type:
- Article
Comparative proteomic profiling reveals mechanisms for early spinal cord vulnerability in CLN1 disease.
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- Scientific Reports, 2020, v. 10, n. 1, p. N.PAG, doi. 10.1038/s41598-020-72075-7
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- Publication type:
- Article
Intravenous administration of scAAV9-Hexb normalizes lifespan and prevents pathology in Sandhoff disease mice.
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- Human Molecular Genetics, 2018, v. 27, n. 6, p. 954, doi. 10.1093/hmg/ddy012
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- Publication type:
- Article
Cathepsin F mutations cause Type B Kufs disease, an adult-onset neuronal ceroid lipofuscinosis.
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- Human Molecular Genetics, 2013, v. 22, n. 7, p. 1417, doi. 10.1093/hmg/dds558
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- Publication type:
- Article
Neurodegeneration and Epilepsy in a Zebrafish Model of CLN3 Disease (Batten Disease).
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- PLoS ONE, 2016, v. 11, n. 6, p. 1, doi. 10.1371/journal.pone.0157365
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- Publication type:
- Article
Intraventricular Enzyme Replacement Improves Disease Phenotypes in a Mouse Model of Late Infantile Neuronal Ceroid Lipofuscinosis.
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- Molecular Therapy, 2008, v. 16, n. 4, p. 649, doi. 10.1038/mt.2008.9
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- Publication type:
- Article
CNS-Directed AAV2-Mediated Gene Therapy Ameliorates Functional Deficits in a Murine Model of Infantile Neuronal Ceroid Lipofuscinosis.
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- Molecular Therapy, 2006, v. 13, n. 3, p. 538, doi. 10.1016/j.ymthe.2005.11.008
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- Publication type:
- Article
Modelling Neurological Diseases in Large Animals: Criteria for Model Selection and Clinical Assessment.
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- Cells (2073-4409), 2022, v. 11, n. 17, p. 2641, doi. 10.3390/cells11172641
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- Publication type:
- Article
Regional Brain Atrophy in Mouse Models of Neuronal Ceroid Lipofuscinosis: A New Rostrocaudal Perspective.
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- Journal of Child Neurology, 2013, v. 28, n. 9, p. 1117, doi. 10.1177/0883073813494479
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- Publication type:
- Article
Brain volume in chronic ketamine users — relationship to sub-threshold psychotic symptoms and relevance to schizophrenia.
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- Psychopharmacology, 2022, v. 239, n. 11, p. 3421, doi. 10.1007/s00213-021-05873-0
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- Article
Spatial and temporal correlation between neuron loss and neuroinflammation in a mouse model of neuronopathic Gaucher disease.
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- Human Molecular Genetics, 2011, v. 20, n. 7, p. 1375, doi. 10.1093/hmg/ddr019
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- Publication type:
- Article
Effects of cis-regulatory variation differ across regions of the adult human brain.
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- Human Molecular Genetics, 2010, v. 19, n. 22, p. 4490, doi. 10.1093/hmg/ddq380
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- Publication type:
- Article
Molecular correlates of axonal and synaptic pathology in mouse models of Batten disease.
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- Human Molecular Genetics, 2009, v. 18, n. 21, p. 4066, doi. 10.1093/hmg/ddp355
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- Publication type:
- Article
An autoantibody inhibitory to glutamic acid decarboxylase in the neurodegenerative disorder Batten disease.
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- Human Molecular Genetics, 2002, v. 11, n. 12, p. 1421, doi. 10.1093/hmg/11.12.1421
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- Publication type:
- Article
An immunohistochemical and stereological analysis of PSI-induced nigral neuronal degeneration in the rat.
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- Journal of Neurochemistry, 2009, v. 109, n. 1, p. 52, doi. 10.1111/j.1471-4159.2009.05956.x
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- Publication type:
- Article
Myelin and Lipid Composition of the Corpus Callosum in Mucopolysaccharidosis Type I Mice.
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- Lipids, 2020, v. 55, n. 6, p. 627, doi. 10.1002/lipd.12261
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- Publication type:
- Article
An Anti-Neuroinflammatory That Targets Dysregulated Glia Enhances the Efficacy of CNS-Directed Gene Therapy in Murine Infantile Neuronal Ceroid Lipofuscinosis.
- Published in:
- Journal of Neuroscience, 2014, v. 34, n. 39, p. 13077, doi. 10.1523/JNEUROSCI.2518-14.2014
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- Publication type:
- Article