Keywords: بیماری باتنت; Lysosomal Storage Disorders; Oxidative stress; Reactive species; Oxidative damage; 4-HNE; 4-hydroxynonenal; 7-KC; 7-ketocholesterol; 8-OHdG; 8-hydroxy-2â²-deoxyguanosine; AGE; advanced glycation end product; BD; Batten disease; Ca2Â +; Â calcium; CAT; ca
مقالات ISI بیماری باتنت (ترجمه نشده)
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Keywords: بیماری باتنت; ERT; enzyme replacement therapy; CLN1; ceroid lipofuscinosis, neuronal-1; CLN2; ceroid lipofuscinosis, neuronal-2; LSD; lysosomal storage disorder; MU-6S-Palm-βGlc; 4-methylumbelliferyl-6-thiopalmitoyl-β-d-glucoside; NCL; neuronal ceroid lipofuscinosis;
Keywords: بیماری باتنت; juvenile neuronal ceroid lipofuscinosis; JNCL; NCL; CLN3; Batten disease; lysosomal storage disease; hippocampus; MRI volumetry
Keywords: بیماری باتنت; batten disease; lysosomal storage disease; retinal degeneration; electroretinogram; pupillary light reflex; optic nerve; ganglion cells; brain;
Paroxysmal sympathetic hyperactivity in Juvenile neuronal ceroid lipofuscinosis (Batten disease)
Keywords: بیماری باتنت; Batten disease; Non epileptic seizure; Neuronal ceroid lipofuscinosis, Paroxysmal sympathetic hyperactivity;
Secretion and function of Cln5 during the early stages of Dictyostelium development
Keywords: بیماری باتنت; Acb; acyl-coenzyme-binding protein; BFA; brefeldin A; bsr; blasticidin resistance; cAMP; cyclic adenosine monophosphate; CTS; cathepsin; CLN; ceroid lipofuscinosis neuronal; CM; conditioned media; CMF; conditioned media factor; CV; contractile vacuole; CQ
Discovery of a CLN7 model of Batten disease in non-human primates
Keywords: بیماری باتنت; CLN7; MFSD8; Late infantile neuronal ceroid lipofuscinosis; Batten disease; Non-human primate; Neurodegeneration; Retinal degeneration; Lysosomal storage disease; Japanese macaque; Large animal model;
Cln3 function is linked to osmoregulation in a Dictyostelium model of Batten disease
Keywords: بیماری باتنت; Neuronal ceroid lipofuscinosis; Batten disease; CLN3; Dictyostelium discoideum; Osmoregulation; RNA sequencing;
Cln5 is secreted and functions as a glycoside hydrolase in Dictyostelium
Keywords: بیماری باتنت; AprA; autocrine proliferation repressor A; CadA; calcium-dependent cell adhesion molecule A; CDC48; cell division cycle protein 48; CfaD; counting factor-associated protein D; CLN5; ceroid lipofuscinosis neuronal 5; CM; conditioned media; CprA; cysteine p
in vivo localization of the neuronal ceroid lipofuscinosis proteins, CLN3 and CLN7, at endogenous expression levels
Keywords: بیماری باتنت; CLN3; CLN7; MFSD8; Localization; Neuronal ceroid lipofuscinosis; Batten disease; Drosophila;
Characterisation of early changes in ovine CLN5 and CLN6 Batten disease neural cultures for the rapid screening of therapeutics
Keywords: بیماری باتنت; Batten disease; Neuronal ceroid lipofuscinosis; CLN5; CLN6; Neuronal cell culture; Gene therapy; Autophagy;
NCLs and ER: A stressful relationship
Keywords: بیماری باتنت; Batten disease; CLN1; CLN3; CLN6; CLN8; ER stress;
Loss of Cln3 impacts protein secretion in the social amoeba Dictyostelium
Keywords: بیماری باتنت; 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
Neuronal ceroid lipofuscinosis associated with an MFSD8 mutation in Chihuahuas
Keywords: بیماری باتنت; Batten disease; Neurodegenerative disease; Retinal degeneration; CLN7; Canine model; Lysosomal storage disease;
Antigen presenting cell abnormalities in the Cln3â/â mouse model of juvenile neuronal ceroid lipofuscinosis
Keywords: بیماری باتنت; 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
Research paperAbnormally increased surface expression of AMPA receptors in the cerebellum, cortex and striatum of Cln3â/â mice
Keywords: بیماری باتنت; 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
Future perspectives: Moving towards NCL treatments
Keywords: بیماری باتنت; Neuronal Ceroid Lipofuscinosis (NCL); Batten disease;
Recent studies of ovine neuronal ceroid lipofuscinoses from BARN, the Batten Animal Research Network
Keywords: بیماری باتنت; Neuronal ceroid lipofuscinoses; NCLs; Batten Disease; CLN5; CLN6; Animal models; Neuroinflammation; Neurodegeneration; Gene therapy; Neural cultures; Neuroinflammatory pathway; Disease associated cardiology;
Experimental therapies in the neuronal ceroid lipofuscinoses
Keywords: بیماری باتنت; Neuronal ceroid lipofuscinosis; Batten disease; Small molecule therapy; Cell therapy; Enzyme replacement therapy; Gene therapy;
Golden Retriever dogs with neuronal ceroid lipofuscinosis have a two-base-pair deletion and frameshift in CLN5
Keywords: بیماری باتنت; Whole genome sequence; Neuronal ceroid lipofuscinosis; Canine model; CLN5 mutation; Batten disease; Lysosomal storage disease; Neurodegeneration;
Cell biology of the NCL proteins: What they do and don't do
Keywords: بیماری باتنت; Neuronal ceroid lipofuscinoses; Batten disease; CLN1-14; NCL proteins;
Valproate-induced hyperammonemia in juvenile ceroid lipofuscinosis (Batten disease)
Keywords: بیماری باتنت; Batten disease; Carnitine; Hyperammonemia; Juvenile ceroid lipofuscinosis; Valproate; Valproate-induced hyperammonemic encephalopathy
A CLN8 nonsense mutation in the whole genome sequence of a mixed breed dog with neuronal ceroid lipofuscinosis and Australian Shepherd ancestry
Keywords: بیماری باتنت; Batten disease; Dog model; CLN8 mutation; Lysosomal storage disease; Retina; Brain;
Amlodipine prevents apoptotic cell death by correction of elevated intracellular calcium in a primary neuronal model of Batten disease (CLN3 disease)
Keywords: بیماری باتنت; CLN; ceroid-lipofuscinosis neuronal (gene symbol); NCL; neuronal ceroid-lipofuscinoses; GFP; green fluorescent protein; siRNA; small inhibiting RNA; Primary neurons; Batten disease; Apoptosis; L-type calcium channel antagonists; Calcium signaling;
Pathogenesis and therapies for infantile neuronal ceroid lipofuscinosis (infantile CLN1 disease)
Keywords: بیماری باتنت; Neuronal ceroid lipofuscinosis; Batten disease; Neurodegeneration; Neuroinflammation; Lysosomal storage disease; Gene therapy
Do females with juvenile ceroid lipofuscinosis (Batten disease) have a more severe disease course? The Danish experience
Keywords: بیماری باتنت; Juvenile neuronal ceroid lipofuscinosis; Batten disease; Outcome; Sex difference; Neurodegenerative
Batten disease is linked to altered expression of mitochondria-related metabolic molecules
Keywords: بیماری باتنت; Batten disease; Mitochondria; ROS
The neuronal ceroid-lipofuscinoses: A historical introduction
Keywords: بیماری باتنت; Neuronal ceroid-lipofuscinosis; Batten disease; History; Neurodegeneration; Ageing; Molecular genetic classification
Juvenile neuronal ceroid lipofuscinosis and education
Keywords: بیماری باتنت; Batten disease; Juvenile neuronal ceroid lipofuscinoses; Education; School; Development
Use of model organisms for the study of neuronal ceroid lipofuscinosis
Keywords: بیماری باتنت; Batten disease; Lysosomal storage disorder; Animal model; Disease mechanism; Pathology; Experimental therapy
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
Keywords: بیماری باتنت; NCL; neuronal ceroid lipofuscinosis; PPT1; palmitoyl-protein thioesterase-1; CLN1; ceroid lipofuscinosis, neuronal-1; GROD; granular osmiophilic deposits; FBS; fetal bovine serum; MU-6S-Palm-βGlc; 4-methylumbelliferyl-6-thiopalmitoyl-β-D-glucoside; PBS;
Selective spatiotemporal patterns of glial activation and neuron loss in the sensory thalamocortical pathways of neuronal ceroid lipofuscinosis 8 mice
Keywords: بیماری باتنت; Batten disease; Stereology; Neurodegeneration; Thalamus; Gliosis; Lysosomal storage; Glial fibrillary acidic protein; Cluster of differentiation 68;
Protective potential of resveratrol against oxidative stress and apoptosis in Batten disease lymphoblast cells
Keywords: بیماری باتنت; Batten disease; Resveratrol; Antioxidant; Apoptosis;
Immunosuppression alters disease severity in juvenile Batten disease mice
Keywords: بیماری باتنت; CLN3; JNCL; Batten disease; Immunosuppression
The specific loss of GnRH-positive neurons from the hypothalamus of sheep with CLN6 neuronal ceroid lipofuscinosis occurs without glial activation and has only minor effects on reproduction
Keywords: بیماری باتنت; Batten disease; Hypothalamic–pituitary–gonadal axis; Sheep model; GnRH neurons; Fertility; Neurodegeneration; Microglia; Astrocytes
A two-dimensional protein fragmentation-proteomic study of neuronal ceroid lipofuscinoses: Identification and characterization of differentially expressed proteins
Keywords: بیماری باتنت; Neuronal ceroid lipofuscinosis; Batten disease; Proteomics; PF2D; Cytoskeleton
Human recombinant palmitoyl-protein thioesterase-1 (PPT1) for preclinical evaluation of enzyme replacement therapy for infantile neuronal ceroid lipofuscinosis
Keywords: بیماری باتنت; Enzyme replacement therapy; Lysosomal storage disorder; Batten disease; Infantile neuronal ceroid lipofuscinosis
Omega-3 and omega-6 fatty acids suppress ER- and oxidative stress in cultured neurons and neuronal progenitor cells from mice lacking PPT1
Keywords: بیماری باتنت; INCL; Palmitoyl-protein thioesterase-1; Batten disease; Neurodegeneration; ER-stress; Oxidative stress; Apoptosis; PUFA
Identifying cellular pathways modulated by Drosophila palmitoyl-protein thioesterase 1 function
Keywords: بیماری باتنت; Neuronal Ceroid Lipofuscinosis; Batten Disease; Palmitoylation; Palmitoyl-protein thioesterase 1; Trafficking; Endocytosis; Drosophila models
Juvenile Neuronal Ceroid Lipofuscinosis (JNCL) and the Eye
Keywords: بیماری باتنت; autofluorescence; Batten disease; ceroid; CLN3; neuronal ceroid ipofuscinosis Ceroid; retinal degeneration
Cerebellar pathology and motor deficits in the palmitoyl protein thioesterase 1-deficient mouse
Keywords: بیماری باتنت; Batten disease; Lysosomal storage disease; Rotarod; Cerebellar mutant; Gliosis; Astrocytes; Silver degeneration staining; Neurodegeneration; GLAST; Glutamine synthetase;
Progressive thalamocortical neuron loss in Cln5 deficient mice: Distinct effects in Finnish variant late infantile NCL
Keywords: بیماری باتنت; Finnish variant late infantile neuronal ceroid lipofuscinosis; Batten disease; CLN5; Thalamocortical neurodegeneration; Lysosomal storage disorder;
Cerebellar defects in a mouse model of juvenile neuronal ceroid lipofuscinosis
Keywords: بیماری باتنت; Batten disease; CLN3; Fastigial; Astrogliosis; Bergmann glia
The transmembrane topology of Batten disease protein CLN3 determined by consensus computational prediction constrained by experimental data
Keywords: بیماری باتنت; CLN3; Batten disease; Transmembrane; Topology; Bioinformatics
A new large animal model of CLN5 neuronal ceroid lipofuscinosis in Borderdale sheep is caused by a nucleotide substitution at a consensus splice site (c.571 + 1G >>> A) leading to excision of exon 3
Keywords: بیماری باتنت; CLN5; NCL; Batten disease; mRNA splicing; Animal model; Borderdale sheep; Lysosomal storage disease
Novel interactions of CLN3 protein link Batten disease to dysregulation of fodrin–Na+, K+ ATPase complex
Keywords: بیماری باتنت; 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: بیماری باتنت; Behavior; Neurodegeneration; Mouse model; Lysosomal storage; Retina; Brain; Batten disease; Electroretinogram; CLN3
Location and connectivity determine GABAergic interneuron survival in the brains of South Hampshire sheep with CLN6 neuronal ceroid lipofuscinosis
Keywords: بیماری باتنت; Batten disease; Neuronal ceroid lipofuscinosis; CLN6; GABAergic interneurons; Ovine model; Lysosomal storage disease
A novel role of the Batten disease gene CLN3: Association with BMP synthesis
Keywords: بیماری باتنت; Neuronal Ceroid Lipofuscinosis; Batten disease; CLN3; Bis(monoacylglycerol)phosphate; Lysobisphosphatidic acid; Detergent-resistant microdomains
In vivo MRI reveals the dynamics of pathological changes in the brains of cathepsin D-deficient mice and correlates changes in manganese-enhanced MRI with microglial activation
Keywords: بیماری باتنت; Batten disease; Brain; Cathepsin D; Magnetic resonance imaging; Mouse