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Cell biology of the NCL proteins: What they do and don't do

Biochimica et biophysica acta. Molecular basis of disease, 2015-10, Vol.1852 (10), p.2242-2255

2015

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Acidified drinking water improves motor function, prevents tremors and changes disease trajectory in Cln2R207X mice, a model of late infantile Batten disease

Scientific reports, 2023-11, Vol.13 (1), p.19229-19229, Article 19229

2023

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The Human Synaptic Vesicle Protein, SV2A, Functions as a Galactose Transporter in Saccharomyces cerevisiae

The Journal of biological chemistry, 2014-11, Vol.289 (48), p.33066-33071

2014

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Role of the Lysosomal Membrane Protein, CLN3, in the Regulation of Cathepsin D Activity

Journal of cellular biochemistry, 2017-11, Vol.118 (11), p.3883-3890

2017

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A tailored mouse model of CLN2 disease: A nonsense mutant for testing personalized therapies

PloS one, 2017-05, Vol.12 (5), p.e0176526-e0176526

2017

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Finding the most appropriate mouse model of juvenile CLN3 (Batten) disease for therapeutic studies: the importance of genetic background and gender

Disease models & mechanisms, 2015-04, Vol.8 (4), p.351-361

2015

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Changes in motor behavior, neuropathology, and gut microbiota of a Batten disease mouse model following administration of acidified drinking water

Scientific reports, 2019-10, Vol.9 (1), p.14962-16, Article 14962

2019

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A novel porcine model of ataxia telangiectasia reproduces neurological features and motor deficits of human disease

Human molecular genetics, 2015-11, Vol.24 (22), p.6473-6484

2015

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A tailored Cln3Q352X mouse model for testing therapeutic interventions in CLN3 Batten disease

Scientific reports, 2020-06, Vol.10 (1), p.10591-10591, Article 10591

2020

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Lack of specificity of antibodies raised against CLN3, the lysosomal/endosomal transmembrane protein mutated in juvenile Batten disease

Bioscience reports, 2017-12, Vol.37 (6)

2017

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Attenuation of AMPA receptor activity improves motor skills in a mouse model of juvenile Batten disease

Experimental neurology, 2008-01, Vol.209 (1), p.288-291

2008

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Location- and sex-specific differences in weight and motor coordination in two commonly used mouse strains

Scientific reports, 2013-07, Vol.3 (1), p.2116, Article 2116

2013

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Tissue-specific variation in nonsense mutant transcript level and drug-induced read-through efficiency in the Cln1(R151X) mouse model of INCL

Journal of cellular and molecular medicine, 2016-02, Vol.20 (2), p.381-385

2016

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Abnormally increased surface expression of AMPA receptors in the cerebellum, cortex and striatum of Cln3−/− mice

Neuroscience letters, 2015-10, Vol.607, p.29-34

2015

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Treatment of the Ppt1–/– Mouse Model of Infantile Neuronal Ceroid Lipofuscinosis With the N-methyl-d-aspartate (NMDA) Receptor Antagonist Memantine

Journal of child neurology, 2013-09, Vol.28 (9), p.1159-1168

2013

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Altered sensitivity of cerebellar granule cells to glutamate receptor overactivation in the Cln3 Δex7/8 -knock-in mouse model of juvenile neuronal ceroid lipofuscinosis

Neurochemistry international, 2011-05, Vol.58 (6), p.648-655

2011

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Antigen presenting cell abnormalities in the Cln3−/− mouse model of juvenile neuronal ceroid lipofuscinosis

Biochimica et biophysica acta, 2016-07, Vol.1862 (7), p.1324-1336

2016

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Decreased sensitivity of palmitoyl protein thioesterase 1-deficient neurons to chemical anoxia

Metabolic brain disease, 2017-02, Vol.32 (1), p.275-279

2017

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Altered sensitivity to excitotoxic cell death and glutamate receptor expression between two commonly studied mouse strains

Journal of neuroscience research, 2010-09, Vol.88 (12), p.2648-2660

2010

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Selectively increased sensitivity of cerebellar granule cells to AMPA receptor-mediated excitotoxicity in a mouse model of Batten disease

Neurobiology of disease, 2006-06, Vol.22 (3), p.575-585

2006

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Altered glutamate receptor function in the cerebellum of the Ppt1−/− mouse, a murine model of infantile neuronal ceroid lipofuscinosis

Journal of neuroscience research, 2012-02, Vol.90 (2), p.367-375

2012

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The acidified drinking water-induced changes in the behavior and gut microbiota of wild-type mice depend on the acidification mode

Scientific reports, 2021-02, Vol.11 (1), p.2877-2877, Article 2877

2021

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Seizure Susceptibility, Phenotype, and Resultant Growth Delay in the nclf and mnd Mouse Models of Neuronal Ceroid Lipofuscinoses

Journal of child neurology, 2013-09, Vol.28 (9), p.1137-1141

2013

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The novel Cln1(R151X) mouse model of infantile neuronal ceroid lipofuscinosis (INCL) for testing nonsense suppression therapy

Human molecular genetics, 2015-01, Vol.24 (1), p.185-196

2015

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Recruitment of active glycogen synthase kinase-3 into neuronal lipid rafts

Biochemical and biophysical research communications, 2006-07, Vol.345 (4), p.1643-1648

2006

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Cell biology of the NCL proteins: What they do and don't do

Acidified drinking water improves motor function, prevents tremors and changes disease trajectory in Cln2R207X mice, a model of late infantile Batten disease

The Human Synaptic Vesicle Protein, SV2A, Functions as a Galactose Transporter in Saccharomyces cerevisiae

Role of the Lysosomal Membrane Protein, CLN3, in the Regulation of Cathepsin D Activity

A tailored mouse model of CLN2 disease: A nonsense mutant for testing personalized therapies

Finding the most appropriate mouse model of juvenile CLN3 (Batten) disease for therapeutic studies: the importance of genetic background and gender

Changes in motor behavior, neuropathology, and gut microbiota of a Batten disease mouse model following administration of acidified drinking water

A novel porcine model of ataxia telangiectasia reproduces neurological features and motor deficits of human disease

A tailored Cln3Q352X mouse model for testing therapeutic interventions in CLN3 Batten disease

Lack of specificity of antibodies raised against CLN3, the lysosomal/endosomal transmembrane protein mutated in juvenile Batten disease

Attenuation of AMPA receptor activity improves motor skills in a mouse model of juvenile Batten disease

Location- and sex-specific differences in weight and motor coordination in two commonly used mouse strains

Tissue-specific variation in nonsense mutant transcript level and drug-induced read-through efficiency in the Cln1(R151X) mouse model of INCL

Abnormally increased surface expression of AMPA receptors in the cerebellum, cortex and striatum of Cln3−/− mice

Treatment of the Ppt1–/– Mouse Model of Infantile Neuronal Ceroid Lipofuscinosis With the N-methyl-d-aspartate (NMDA) Receptor Antagonist Memantine

Altered sensitivity of cerebellar granule cells to glutamate receptor overactivation in the Cln3 Δex7/8 -knock-in mouse model of juvenile neuronal ceroid lipofuscinosis

Antigen presenting cell abnormalities in the Cln3−/− mouse model of juvenile neuronal ceroid lipofuscinosis

Decreased sensitivity of palmitoyl protein thioesterase 1-deficient neurons to chemical anoxia

Altered sensitivity to excitotoxic cell death and glutamate receptor expression between two commonly studied mouse strains

Selectively increased sensitivity of cerebellar granule cells to AMPA receptor-mediated excitotoxicity in a mouse model of Batten disease

Altered glutamate receptor function in the cerebellum of the Ppt1−/− mouse, a murine model of infantile neuronal ceroid lipofuscinosis

The acidified drinking water-induced changes in the behavior and gut microbiota of wild-type mice depend on the acidification mode

Seizure Susceptibility, Phenotype, and Resultant Growth Delay in the nclf and mnd Mouse Models of Neuronal Ceroid Lipofuscinoses

The novel Cln1(R151X) mouse model of infantile neuronal ceroid lipofuscinosis (INCL) for testing nonsense suppression therapy

Recruitment of active glycogen synthase kinase-3 into neuronal lipid rafts

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