Keywords: Ethanol fermentation; Brewer's yeast; Saccharomyces pastorianus; Quiescent state; G0 entry; RIM15; CLN3;
مقالات ISI (ترجمه نشده)
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Keywords: juvenile neuronal ceroid lipofuscinosis; JNCL; NCL; CLN3; Batten disease; lysosomal storage disease; hippocampus; MRI volumetry
Modulating membrane fluidity corrects Batten disease phenotypes in vitro and in vivo
Keywords: Neuronal ceroid lipofuscinoses; JNCL; CLN3; Cav-1; Cdc42; Blood-brain barrier; Carbenoxolone; Membrane fluidity;
Cln3 function is linked to osmoregulation in a Dictyostelium model of Batten disease
Keywords: Neuronal ceroid lipofuscinosis; Batten disease; CLN3; Dictyostelium discoideum; Osmoregulation; RNA sequencing;
in vivo localization of the neuronal ceroid lipofuscinosis proteins, CLN3 and CLN7, at endogenous expression levels
Keywords: CLN3; CLN7; MFSD8; Localization; Neuronal ceroid lipofuscinosis; Batten disease; Drosophila;
NCLs and ER: A stressful relationship
Keywords: Batten disease; CLN1; CLN3; CLN6; CLN8; ER stress;
Loss of Cln3 impacts protein secretion in the social amoeba Dictyostelium
Keywords: AprA; autocrine proliferation repressor A; CfaD; counting factor-associated protein D; CF; counting factor; CLN3; ceroid lipofuscinosis neuronal 3; CM; conditioned media; CMF; conditioned media factor; CsbA; contact site B protein A; CV; contractile vacuo
TORC1 activity is partially reduced under nitrogen starvation conditions in sake yeast Kyokai no. 7, Saccharomyces cerevisiae
Keywords: Sake yeasts; TORC1 activity; Nitrogen catabolite repression; Cln3; Autophagy
Antigen presenting cell abnormalities in the Cln3â/â mouse model of juvenile neuronal ceroid lipofuscinosis
Keywords: APCs; antigen presenting cells; APhC; allophycocyanin; BM; bone marrow; BMCCs; bone marrow cultured cells; BM-DCs; bone marrow dendritic cells; BM-MÏ; bone marrow macrophages; BSA; bovine serum albumin; CHX; cycloheximide; CTB; cholera toxin B; DCs; dend
A novel mutation of CLN3 associated with delayed-classic juvenile ceroid lipofuscinois and autophagic vacuolar myopathy
Keywords: Juvenile neuronal ceroid lipofuscinosis (JNCL); Delayed-classic JNCL; CLN3; Autophagic vacuolar myopathy
Research paperAbnormally increased surface expression of AMPA receptors in the cerebellum, cortex and striatum of Cln3â/â mice
Keywords: AMPA; α-amino-3-hydroxy-5-methyl-4-isoxazolepropionate; NMDA; N-methyl-d-aspartate; BS3; bis[sulfosuccinimidyl] suberate; ACSF; artificial cerebrospinal fluid; Juvenile neuronal ceroid lipofuscinosis; Batten disease; CLN3; Glutamate receptor; AMPA recept
Effect of CLN3 silencing by RNA interference on the proliferation and apoptosis of human colorectal cancer cells
Keywords: Colorectal cancer; CLN3; RNA interference; Proliferation; Apoptosis
The Batten disease gene CLN3 confers resistance to endoplasmic reticulum stress induced by tunicamycin
Keywords: CLN3; Endoplasmic reticulum (ER) stress; JNCL; Tunicamycin;
Age-dependent therapeutic effect of memantine in a mouse model of juvenile Batten disease
Keywords: Juvenile Batten disease; Neuronal ceroid lipofuscinoses; Cln3; NMDA receptor; AMPA receptor; Rotarod; AMPA; α-amino-3-hydroxy-5-methyl-4-isoxazolepropionate; NMDA; N-methyl-d-aspartate; WT; wild type; DPBS; Dulbecco's phosphate-buffered saline; TBS; Tris
Immunosuppression alters disease severity in juvenile Batten disease mice
Keywords: CLN3; JNCL; Batten disease; Immunosuppression
Temporary inhibition of AMPA receptors induces a prolonged improvement of motor performance in a mouse model of juvenile Batten disease
Keywords: Juvenile Batten disease; Neuronal ceroid lipofuscinoses; Cln3; AMPA receptor; EGIS-8332; Rotarod; AMPA; α-amino-3-hydroxy-5-methyl-4-isoxazolepropionate; NMDA; N-methyl-d-aspartate; WT; wild type; DPBS; Dulbecco's phosphate-buffered saline; TBS; tris b
Ethanol fermentation driven by elevated expression of the G1 cyclin gene CLN3 in sake yeast
Keywords: Sake yeast; Saccharomyces cerevisiae; Ethanol fermentation; CalMorph system; G1 cyclin; CLN3; whi mutants; G1/S transition
A knock-in reporter mouse model for Batten disease reveals predominant expression of Cln3 in visual, limbic and subcortical motor structures
Keywords: CLN3; Neuronal ceroid lipofuscinosis; JNCL; Thalamus; Hippocampal formation; Red nucleus; Reticular formation; Cerebellum; Retina; Mouse model
Neuropeptide changes and neuroactive amino acids in CSF from humans and sheep with neuronal ceroid lipofuscinoses (NCLs, Batten disease)
Keywords: Neuron specific enolase; Tau; Brain specific proteins; IGF-1; GABA; CSF; CLN2; CLN3; CLN6; Neurodegeneration; Biomarkers
Juvenile Neuronal Ceroid Lipofuscinosis (JNCL) and the Eye
Keywords: autofluorescence; Batten disease; ceroid; CLN3; neuronal ceroid ipofuscinosis Ceroid; retinal degeneration
Molecular networks and system-level properties
Keywords: Systems biology; Cell cycle; G1/S transition; Cki; Sic1; Far1; Cln3; CK2; Robustness; Emergent property; Network
Cerebellar defects in a mouse model of juvenile neuronal ceroid lipofuscinosis
Keywords: Batten disease; CLN3; Fastigial; Astrogliosis; Bergmann glia
Attenuation of AMPA receptor activity improves motor skills in a mouse model of juvenile Batten disease
Keywords: Juvenile Batten disease; Cln3; Cln3Îex1-6; Mouse model; Motor skills; AMPA receptor antagonist; EGIS-8332;
The transmembrane topology of Batten disease protein CLN3 determined by consensus computational prediction constrained by experimental data
Keywords: CLN3; Batten disease; Transmembrane; Topology; Bioinformatics
Novel interactions of CLN3 protein link Batten disease to dysregulation of fodrin–Na+, K+ ATPase complex
Keywords: Batten disease; JNCL; CLN3; Fodrin; Na+, K+ ATPase; TIRF microscopy; Primary neurons; Ouabain-induced endocytosis
Phenotypic characterization of a mouse model of juvenile neuronal ceroid lipofuscinosis
Keywords: Behavior; Neurodegeneration; Mouse model; Lysosomal storage; Retina; Brain; Batten disease; Electroretinogram; CLN3
A novel role of the Batten disease gene CLN3: Association with BMP synthesis
Keywords: Neuronal Ceroid Lipofuscinosis; Batten disease; CLN3; Bis(monoacylglycerol)phosphate; Lysobisphosphatidic acid; Detergent-resistant microdomains
Alterations in striatal dopamine catabolism precede loss of substantia nigra neurons in a mouse model of juvenile neuronal ceroid lipofuscinosis
Keywords: Batten disease; Catechol-O-methyltransferase; CLN3; Dopamine; Oxidative stress
Homogeneous PCR nucleobase quenching assays to detect four mutations that cause neuronal ceroid lipofuscinosis: T75P and R151X in CLN1, and IVS5-1G > C and R208X in CLN2
Keywords: CLN1; CLN2; CLN3; Neuronal ceroid lipofuscinosis; Batten disease; LightCycler; Nucleobase quenching; Mutation
Visual deficits in a mouse model of Batten disease are the result of optic nerve degeneration and loss of dorsal lateral geniculate thalamic neurons
Keywords: Juvenile neuronal ceroid lipofuscinosis; CLN3; Lateral geniculate nucleus; Thalamus; Axonal transport
Novel CLN3 mutation predicted to cause complete loss of protein function does not modify the classical JNCL phenotype
Keywords: Batten disease; CLN3; Novel mutation; JNCL; Juvenile Neuronal Ceroid Lipofuscinosis;
Elevation of Hook1 in a disease model of Batten disease does not affect a novel interaction between Ankyrin G and Hook1
Keywords: Ankyrin G; Hook1; CLN3; Cytoskeleton; Protein trafficking; Batten disease;
Thalamocortical neuron loss and localized astrocytosis in the Cln3Îex7/8 knock-in mouse model of Batten disease
Keywords: Batten disease; Juvenile neuronal ceroid lipofuscinosis; JNCL; CLN3; Thalamocortical degeneration; Astrocytosis; Lysosomal storage disorder;