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Search results 1 to 100 out of 362 for Cln3

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0.02s
Type Details Score
Gene
Type: gene
Organism: human
Gene
Type: gene
Organism: zebrafish
Gene
Type: gene
Organism: cattle
Gene
Type: gene
Organism: macaque, rhesus
Gene
Type: gene
Organism: rat
Gene
Type: gene
Organism: dog, domestic
Gene
Type: gene
Organism: frog, western clawed
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Publication
First Author: Laqtom NN
Year: 2022
Journal: Nature
Title: CLN3 is required for the clearance of glycerophosphodiesters from lysosomes.
Volume: 609
Issue: 7929
Pages: 1005-1011
Publication
First Author: Seigel GM
Year: 2002
Journal: Mol Cell Neurosci
Title: Retinal pathology and function in a Cln3 knockout mouse model of juvenile Neuronal Ceroid Lipofuscinosis (batten disease).
Volume: 19
Issue: 4
Pages: 515-27
Publication  
First Author: Johnson TB
Year: 2023
Journal: Front Genet
Title: Early postnatal administration of an AAV9 gene therapy is safe and efficacious in CLN3 disease.
Volume: 14
Pages: 1118649
Publication
First Author: Langin L
Year: 2020
Journal: Sci Rep
Title: A tailored Cln3Q352X mouse model for testing therapeutic interventions in CLN3 Batten disease.
Volume: 10
Issue: 1
Pages: 10591
Publication
First Author: Getty AL
Year: 2011
Journal: Exp Cell Res
Title: A novel interaction of CLN3 with nonmuscle myosin-IIB and defects in cell motility of Cln3(-/-) cells.
Volume: 317
Issue: 1
Pages: 51-69
Publication
First Author: Centa JL
Year: 2020
Journal: Nat Med
Title: Therapeutic efficacy of antisense oligonucleotides in mouse models of CLN3 Batten disease.
Volume: 26
Issue: 9
Pages: 1444-1451
Publication
First Author: Taschner PE
Year: 1997
Journal: Neuropediatrics
Title: Cross-species homology of the CLN3 gene.
Volume: 28
Issue: 1
Pages: 18-20
Publication
First Author: Mitchison HM
Year: 1999
Journal: Neurobiol Dis
Title: Targeted disruption of the Cln3 gene provides a mouse model for Batten disease. The Batten Mouse Model Consortium [corrected].
Volume: 6
Issue: 5
Pages: 321-34
Publication
First Author: Tecedor L
Year: 2013
Journal: J Neurosci
Title: CLN3 loss disturbs membrane microdomain properties and protein transport in brain endothelial cells.
Volume: 33
Issue: 46
Pages: 18065-79
Publication
First Author: Chang JW
Year: 2007
Journal: Hum Mol Genet
Title: Neuronal vulnerability of CLN3 deletion to calcium-induced cytotoxicity is mediated by calsenilin.
Volume: 16
Issue: 3
Pages: 317-26
Publication
First Author: Ratajczak E
Year: 2014
Journal: PLoS One
Title: FRET-assisted determination of CLN3 membrane topology.
Volume: 9
Issue: 7
Pages: e102593
Publication
First Author: Ding SL
Year: 2011
Journal: Neurobiol Dis
Title: A knock-in reporter mouse model for Batten disease reveals predominant expression of Cln3 in visual, limbic and subcortical motor structures.
Volume: 41
Issue: 2
Pages: 237-48
Publication
First Author: Kovács AD
Year: 2015
Journal: Dis Model Mech
Title: Finding the most appropriate mouse model of juvenile CLN3 (Batten) disease for therapeutic studies: the importance of genetic background and gender.
Volume: 8
Issue: 4
Pages: 351-61
Publication
First Author: Uusi-Rauva K
Year: 2008
Journal: Exp Cell Res
Title: Novel interactions of CLN3 protein link Batten disease to dysregulation of fodrin-Na+, K+ ATPase complex.
Volume: 314
Issue: 15
Pages: 2895-905
Publication  
First Author: Tarczyluk-Wells MA
Year: 2019
Journal: Front Neurol
Title: Combined Anti-inflammatory and Neuroprotective Treatments Have the Potential to Impact Disease Phenotypes in Cln3 -/- Mice.
Volume: 10
Pages: 963
Publication
First Author: Puranam KL
Year: 1999
Journal: Mol Genet Metab
Title: CLN3 defines a novel antiapoptotic pathway operative in neurodegeneration and mediated by ceramide.
Volume: 66
Issue: 4
Pages: 294-308
Publication
First Author: Schultz ML
Year: 2014
Journal: PLoS One
Title: CLN3 deficient cells display defects in the ARF1-Cdc42 pathway and actin-dependent events.
Volume: 9
Issue: 5
Pages: e96647
Publication
First Author: Zhong N
Year: 1998
Journal: Hum Genet
Title: Molecular screening of Batten disease: identification of a missense mutation (E295K) in the CLN3 gene.
Volume: 102
Issue: 1
Pages: 57-62
Publication  
First Author: El-Sitt S
Year: 2019
Journal: Front Neurol
Title: Developmental Comparison of Ceramide in Wild-Type and Cln3 Δex7/8 Mouse Brains and Sera.
Volume: 10
Pages: 128
Publication
First Author: Oetjen S
Year: 2016
Journal: J Neurochem
Title: Revisiting the neuronal localization and trafficking of CLN3 in juvenile neuronal ceroid lipofuscinosis.
Volume: 139
Issue: 3
Pages: 456-470
Publication
First Author: Klein M
Year: 2022
Journal: Autophagy
Title: Converging roles of PSENEN/PEN2 and CLN3 in the autophagy-lysosome system.
Volume: 18
Issue: 9
Pages: 2068-2085
Publication
First Author: Luiro K
Year: 2001
Journal: Hum Mol Genet
Title: CLN3 protein is targeted to neuronal synapses but excluded from synaptic vesicles: new clues to Batten disease.
Volume: 10
Issue: 19
Pages: 2123-31
Publication
First Author: Greene ND
Year: 1999
Journal: Mol Genet Metab
Title: A murine model for juvenile NCL: gene targeting of mouse Cln3.
Volume: 66
Issue: 4
Pages: 309-13
Publication
First Author: Weimer JM
Year: 2006
Journal: Neurobiol Dis
Title: Visual deficits in a mouse model of Batten disease are the result of optic nerve degeneration and loss of dorsal lateral geniculate thalamic neurons.
Volume: 22
Issue: 2
Pages: 284-93
Publication
First Author: Nyame K
Year: 2024
Journal: Mol Cell
Title: Glycerophosphodiesters inhibit lysosomal phospholipid catabolism in Batten disease.
Volume: 84
Issue: 7
Pages: 1354-1364.e9
Publication
First Author: Pontikis CC
Year: 2004
Journal: Brain Res
Title: Late onset neurodegeneration in the Cln3-/- mouse model of juvenile neuronal ceroid lipofuscinosis is preceded by low level glial activation.
Volume: 1023
Issue: 2
Pages: 231-42
Publication
First Author: Narayan SB
Year: 2008
Journal: Mol Genet Metab
Title: Intermediate levels of neuronal palmitoyl-protein Delta-9 desaturase in heterozygotes for murine Batten disease.
Volume: 93
Issue: 1
Pages: 89-91
Publication
First Author: Kovács AD
Year: 2006
Journal: Neurobiol Dis
Title: Selectively increased sensitivity of cerebellar granule cells to AMPA receptor-mediated excitotoxicity in a mouse model of Batten disease.
Volume: 22
Issue: 3
Pages: 575-85
Publication    
First Author: Grünewald B
Year: 2017
Journal: Elife
Title: Defective synaptic transmission causes disease signs in a mouse model of juvenile neuronal ceroid lipofuscinosis.
Volume: 6
Publication
First Author: Chan CH
Year: 2009
Journal: Neuropathol Appl Neurobiol
Title: Altered arginine metabolism in the central nervous system (CNS) of the Cln3-/- mouse model of juvenile Batten disease.
Volume: 35
Issue: 2
Pages: 189-207
Publication
First Author: Wavre-Shapton ST
Year: 2015
Journal: Hum Mol Genet
Title: Photoreceptor phagosome processing defects and disturbed autophagy in retinal pigment epithelium of Cln3Δex1-6 mice modelling juvenile neuronal ceroid lipofuscinosis (Batten disease).
Volume: 24
Issue: 24
Pages: 7060-74
Publication  
First Author: Studniarczyk D
Year: 2018
Journal: eNeuro
Title: Altered Cerebellar Short-Term Plasticity but No Change in Postsynaptic AMPA-Type Glutamate Receptors in a Mouse Model of Juvenile Batten Disease.
Volume: 5
Issue: 2
Publication
First Author: Kovács AD
Year: 2012
Journal: Neuropharmacology
Title: Age-dependent therapeutic effect of memantine in a mouse model of juvenile Batten disease.
Volume: 63
Issue: 5
Pages: 769-75
Publication
First Author: Pears MR
Year: 2005
Journal: J Biol Chem
Title: High resolution 1H NMR-based metabolomics indicates a neurotransmitter cycling deficit in cerebral tissue from a mouse model of Batten disease.
Volume: 280
Issue: 52
Pages: 42508-14
Publication
First Author: Johnson TB
Year: 2019
Journal: Sci Rep
Title: Changes in motor behavior, neuropathology, and gut microbiota of a Batten disease mouse model following administration of acidified drinking water.
Volume: 9
Issue: 1
Pages: 14962
Publication
First Author: Llavero Hurtado M
Year: 2017
Journal: Sci Rep
Title: Proteomic mapping of differentially vulnerable pre-synaptic populations identifies regulators of neuronal stability in vivo.
Volume: 7
Issue: 1
Pages: 12412
Publication  
First Author: Weimer JM
Year: 2009
Journal: Brain Res
Title: Cerebellar defects in a mouse model of juvenile neuronal ceroid lipofuscinosis.
Volume: 1266
Pages: 93-107
Publication
First Author: Brooks AI
Year: 2003
Journal: Mol Genet Metab
Title: Functional categorization of gene expression changes in the cerebellum of a Cln3-knockout mouse model for Batten disease.
Volume: 78
Issue: 1
Pages: 17-30
Publication
First Author: Kriscenski-Perry E
Year: 2002
Journal: Epilepsia
Title: Altered flurothyl seizure induction latency, phenotype, and subsequent mortality in a mouse model of juvenile neuronal ceroid lipofuscinosis/batten disease.
Volume: 43
Issue: 10
Pages: 1137-40
Publication
First Author: Kovács AD
Year: 2008
Journal: Exp Neurol
Title: Attenuation of AMPA receptor activity improves motor skills in a mouse model of juvenile Batten disease.
Volume: 209
Issue: 1
Pages: 288-91
Publication  
First Author: Weimer JM
Year: 2007
Journal: Brain Res
Title: Alterations in striatal dopamine catabolism precede loss of substantia nigra neurons in a mouse model of juvenile neuronal ceroid lipofuscinosis.
Volume: 1162
Pages: 98-112
Publication
First Author: Chattopadhyay S
Year: 2002
Journal: Hum Mol Genet
Title: An autoantibody inhibitory to glutamic acid decarboxylase in the neurodegenerative disorder Batten disease.
Volume: 11
Issue: 12
Pages: 1421-31
Publication
First Author: Sappington RM
Year: 2003
Journal: Invest Ophthalmol Vis Sci
Title: Optic nerve degeneration in a murine model of juvenile ceroid lipofuscinosis.
Volume: 44
Issue: 9
Pages: 3725-31
Publication
First Author: Chandrachud U
Year: 2015
Journal: J Biol Chem
Title: Unbiased Cell-based Screening in a Neuronal Cell Model of Batten Disease Highlights an Interaction between Ca2+ Homeostasis, Autophagy, and CLN3 Protein Function.
Volume: 290
Issue: 23
Pages: 14361-80
Publication
First Author: Chattopadhyay S
Year: 2004
Journal: Invest Ophthalmol Vis Sci
Title: Altered gene expression in the eye of a mouse model for batten disease.
Volume: 45
Issue: 9
Pages: 2893-905
Publication
First Author: Castaneda JA
Year: 2008
Journal: Neurobiol Dis
Title: Identification of alpha-fetoprotein as an autoantigen in juvenile Batten disease.
Volume: 29
Issue: 1
Pages: 92-102
Publication
First Author: Pearce DA
Year: 2003
Journal: Clin Chim Acta
Title: Altered amino acid levels in sera of a mouse model for juvenile neuronal ceroid lipofuscinoses.
Volume: 332
Issue: 1-2
Pages: 145-8
Publication
First Author: Lim MJ
Year: 2007
Journal: Neurobiol Dis
Title: IgG entry and deposition are components of the neuroimmune response in Batten disease.
Volume: 25
Issue: 2
Pages: 239-51
Publication
First Author: Pontikis CC
Year: 2005
Journal: Neurobiol Dis
Title: Thalamocortical neuron loss and localized astrocytosis in the Cln3Deltaex7/8 knock-in mouse model of Batten disease.
Volume: 20
Issue: 3
Pages: 823-36
Publication
First Author: Fabritius AL
Year: 2014
Journal: Exp Mol Pathol
Title: Neuronal ceroid lipofuscinosis genes, CLN2, CLN3 and CLN5 are spatially and temporally co-expressed in a developing mouse brain.
Volume: 97
Issue: 3
Pages: 484-91
Publication
First Author: Makoukji J
Year: 2020
Journal: PLoS One
Title: Sex differences in gene expression with galactosylceramide treatment in Cln3Δex7/8 mice.
Volume: 15
Issue: 10
Pages: e0239537
Publication
First Author: Osório NS
Year: 2009
Journal: Genes Brain Behav
Title: Neurodevelopmental delay in the Cln3Deltaex7/8 mouse model for Batten disease.
Volume: 8
Issue: 3
Pages: 337-45
Publication
First Author: Schmidtke C
Year: 2019
Journal: J Biol Chem
Title: Lysosomal proteome analysis reveals that CLN3-defective cells have multiple enzyme deficiencies associated with changes in intracellular trafficking.
Volume: 294
Issue: 24
Pages: 9592-9604
Publication
First Author: Zhong Y
Year: 2020
Journal: Biochim Biophys Acta Mol Basis Dis
Title: Loss of CLN3, the gene mutated in juvenile neuronal ceroid lipofuscinosis, leads to metabolic impairment and autophagy induction in retinal pigment epithelium.
Volume: 1866
Issue: 10
Pages: 165883
Publication
First Author: Katz ML
Year: 1999
Journal: J Neurosci Res
Title: A mouse gene knockout model for juvenile ceroid-lipofuscinosis (Batten disease).
Volume: 57
Issue: 4
Pages: 551-6
Publication
First Author: Katz ML
Year: 2008
Journal: Neurobiol Dis
Title: Phenotypic characterization of a mouse model of juvenile neuronal ceroid lipofuscinosis.
Volume: 29
Issue: 2
Pages: 242-53
Publication
First Author: Weimer JM
Year: 2005
Journal: Biochem Biophys Res Commun
Title: Elevation of Hook1 in a disease model of Batten disease does not affect a novel interaction between Ankyrin G and Hook1.
Volume: 330
Issue: 4
Pages: 1176-81
Publication
First Author: Eliason SL
Year: 2007
Journal: J Neurosci
Title: A knock-in reporter model of Batten disease.
Volume: 27
Issue: 37
Pages: 9826-34
Publication
First Author: Stein CS
Year: 2010
Journal: Am J Physiol Cell Physiol
Title: Osmoregulation of ceroid neuronal lipofuscinosis type 3 in the renal medulla.
Volume: 298
Issue: 6
Pages: C1388-400
Publication  
First Author: Palmieri M
Year: 2017
Journal: Nat Commun
Title: mTORC1-independent TFEB activation via Akt inhibition promotes cellular clearance in neurodegenerative storage diseases.
Volume: 8
Pages: 14338
Publication  
First Author: Burkovetskaya M
Year: 2017
Journal: Neurobiol Dis
Title: Age-dependent alterations in neuronal activity in the hippocampus and visual cortex in a mouse model of Juvenile Neuronal Ceroid Lipofuscinosis (CLN3).
Volume: 100
Pages: 19-29
Publication
First Author: Timm D
Year: 2018
Journal: PLoS One
Title: Searching for novel biomarkers using a mouse model of CLN3-Batten disease.
Volume: 13
Issue: 8
Pages: e0201470
Publication
First Author: Burkovetskaya M
Year: 2014
Journal: PLoS One
Title: Evidence for aberrant astrocyte hemichannel activity in Juvenile Neuronal Ceroid Lipofuscinosis (JNCL).
Volume: 9
Issue: 4
Pages: e95023
Publication  
First Author: Schultz ML
Year: 2018
Journal: Neurobiol Dis
Title: Modulating membrane fluidity corrects Batten disease phenotypes in vitro and in vivo.
Volume: 115
Pages: 182-193
Publication
First Author: Elshatory Y
Year: 2003
Journal: FEBS Lett
Title: Early changes in gene expression in two models of Batten disease.
Volume: 538
Issue: 1-3
Pages: 207-12
Publication  
First Author: Ahrens-Nicklas RC
Year: 2019
Journal: JCI Insight
Title: Neuronal network dysfunction precedes storage and neurodegeneration in a lysosomal storage disorder.
Volume: 4
Issue: 21
Publication
First Author: Pohl S
Year: 2007
Journal: J Neurochem
Title: Increased expression of lysosomal acid phosphatase in CLN3-defective cells and mouse brain tissue.
Volume: 103
Issue: 6
Pages: 2177-88
Publication  
First Author: Dannhausen K
Year: 2018
Journal: Dis Model Mech
Title: Immunomodulation with minocycline rescues retinal degeneration in juvenile neuronal ceroid lipofuscinosis mice highly susceptible to light damage.
Volume: 11
Issue: 9
Publication
First Author: Cao Y
Year: 2006
Journal: J Biol Chem
Title: Autophagy is disrupted in a knock-in mouse model of juvenile neuronal ceroid lipofuscinosis.
Volume: 281
Issue: 29
Pages: 20483-93
Publication
First Author: Staropoli JF
Year: 2012
Journal: PLoS One
Title: Large-scale phenotyping of an accurate genetic mouse model of JNCL identifies novel early pathology outside the central nervous system.
Volume: 7
Issue: 6
Pages: e38310
Publication
First Author: Pedram K
Year: 2022
Journal: Proc Natl Acad Sci U S A
Title: Lysosomal cathepsin D mediates endogenous mucin glycodomain catabolism in mammals.
Volume: 119
Issue: 39
Pages: e2117105119
Publication
First Author: Kovács AD
Year: 2023
Journal: Sci Rep
Title: Acidified drinking water improves motor function, prevents tremors and changes disease trajectory in Cln2(R207X) mice, a model of late infantile Batten disease.
Volume: 13
Issue: 1
Pages: 19229
Publication  
First Author: Fossale E
Year: 2004
Journal: BMC Neurosci
Title: Membrane trafficking and mitochondrial abnormalities precede subunit c deposition in a cerebellar cell model of juvenile neuronal ceroid lipofuscinosis.
Volume: 5
Pages: 57
Publication
First Author: Tardy C
Year: 2009
Journal: Biochim Biophys Acta
Title: Palmitoyl protein thioesterase 1 modulates tumor necrosis factor alpha-induced apoptosis.
Volume: 1793
Issue: 7
Pages: 1250-8
Publication
First Author: Hersrud SL
Year: 2016
Journal: Biochim Biophys Acta
Title: Antigen presenting cell abnormalities in the Cln3(-/-) mouse model of juvenile neuronal ceroid lipofuscinosis.
Volume: 1862
Issue: 7
Pages: 1324-36
Publication
First Author: Groh J
Year: 2016
Journal: Glia
Title: Sialoadhesin promotes neuroinflammation-related disease progression in two mouse models of CLN disease.
Volume: 64
Issue: 5
Pages: 792-809
Publication
First Author: Cotman SL
Year: 2002
Journal: Hum Mol Genet
Title: Cln3(Deltaex7/8) knock-in mice with the common JNCL mutation exhibit progressive neurologic disease that begins before birth.
Volume: 11
Issue: 22
Pages: 2709-21
Publication
First Author: Burkovetskaya M
Year: 2019
Journal: J Neurochem
Title: Caspase 1 activity influences juvenile Batten disease (CLN3) pathogenesis.
Volume: 148
Issue: 5
Pages: 652-668
Publication
First Author: Appu AP
Year: 2019
Journal: J Inherit Metab Dis
Title: Cln3-mutations underlying juvenile neuronal ceroid lipofuscinosis cause significantly reduced levels of Palmitoyl-protein thioesterases-1 (Ppt1)-protein and Ppt1-enzyme activity in the lysosome.
Volume: 42
Issue: 5
Pages: 944-954
Publication
First Author: Wendt KD
Year: 2005
Journal: Behav Brain Res
Title: Behavioral assessment in mouse models of neuronal ceroid lipofuscinosis using a light-cued T-maze.
Volume: 161
Issue: 2
Pages: 175-82
Publication
First Author: Koike M
Year: 2013
Journal: Eur J Neurosci
Title: Differences in expression patterns of cathepsin C/dipeptidyl peptidase I in normal, pathological and aged mouse central nervous system.
Volume: 37
Issue: 5
Pages: 816-30
Publication
First Author: Song JW
Year: 2008
Journal: J Neurosci
Title: Lysosomal activity associated with developmental axon pruning.
Volume: 28
Issue: 36
Pages: 8993-9001
Publication
First Author: Lee RL
Year: 1996
Journal: Genomics
Title: Isolation and chromosomal mapping of a mouse homolog of the Batten disease gene CLN3.
Volume: 35
Issue: 3
Pages: 617-9
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus pahari