Selectivity and types of cell death in the neuronal ceroid lipofuscinoses (Q35681068)

9 August 2017

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title

Selectivity and types of cell death in the neuronal ceroid lipofuscinoses (English)

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9 August 2017

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Hannah M Mitchison

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9 August 2017

Ming J Lim

series ordinal

2

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9 August 2017

Jonathan D Cooper

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3

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9 August 2017

language of work or name

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1 January 2004

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9 August 2017

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9 August 2017

14

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9 August 2017

1

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9 August 2017

86-96

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Palmitoyl protein thioesterase 1 is targeted to the axons in neurons

9 August 2017

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

Lysosomal degradation of cholecystokinin-(29-33)-amide in mouse brain is dependent on tripeptidyl peptidase-I: implications for the degradation and storage of peptides in classical late-infantile neuronal ceroid lipofuscinosis

21 January 2018

1 reference

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The development of behavioral abnormalities in the motor neuron degeneration (mnd) mouse

21 January 2018

1 reference

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Onset and progression of motor deficits in motor neuron degeneration (mnd) mice are unaltered by the glycine/NMDA receptor antagonist L-701,324 or the MAO-B inhibitor R(-)-deprenyl

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

Loss of pigment-laden stellate cells: a severe alteration of the isocortex in juvenile neuronal ceroid-lipofuscinosis

21 January 2018

1 reference

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Pigmentoarchitectonic pathology of the isocortex in juvenile neuronal ceroid-lipofuscinosis: Axonal enlargements in layer IIIab and cell loss in layer V

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

Motor neuron degeneration of mice is a model of neuronal ceroid lipofuscinosis (Batten's disease)

21 January 2018

1 reference

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Neuronal ceroid lipofuscinosis (nclf), a new disorder of the mouse linked to chromosome 9

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

An autoantibody inhibitory to glutamic acid decarboxylase in the neurodegenerative disorder Batten disease

21 January 2018

1 reference

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An autoantibody to GAD65 in sera of patients with juvenile neuronal ceroid lipofuscinoses.

21 January 2018

1 reference

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Neuronal ceroid lipofuscinosis in Merino sheep.

21 January 2018

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Progress towards understanding the neurobiology of Batten disease or neuronal ceroid lipofuscinosis

21 January 2018

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https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

Molecular genetics of palmitoyl-protein thioesterase deficiency in the U.S.

21 January 2018

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https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

Altered GABAergic function accompanies hippocampal degeneration in mice lacking ClC-3 voltage-gated chloride channels

21 January 2018

1 reference

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Calretinin and calbindin D-28k, but not parvalbumin protect against glutamate-induced delayed excitotoxicity in transfected N18-RE 105 neuroblastoma-retina hybrid cells.

21 January 2018

1 reference

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Mutations in a novel CLN6-encoded transmembrane protein cause variant neuronal ceroid lipofuscinosis in man and mouse

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

Microglia: Intrinsic immuneffector cell of the brain

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

Progress in neuropathology of the neuronal ceroid lipofuscinoses

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

High resolution MRI reveals global changes in brains of Cln3 mutant mice.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

Disruption of PPT1 or PPT2 causes neuronal ceroid lipofuscinosis in knockout mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

Disruption ofPPT2in mice causes an unusual lysosomal storage disorder with neurovisceral features

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

Infantile type of so-called neuronal ceroid-lipofuscinosis. Histological and electron microscopic studies

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

Infantile type of so-called neuronal ceroid-lipofuscinosis. 2. Morphological and biochemical studies

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

Hippocampal lesions in the neuronal ceroid lipofuscinoses.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

The neuronal ceroid-lipofuscinoses

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

Northern epilepsy: a novel form of neuronal ceroid-lipofuscinosis.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

Neuronal ceroid lipofuscinoses caused by defects in soluble lysosomal enzymes (CLN1 and CLN2)

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

Disease-specific pathology in neurons cultured from sheep affected with ceroid lipofuscinosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

Isolation of a novel gene underlying batten disease, CLN3

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

An animal model of the infantile type of neuronal ceroid-lipofuscinosis.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

Blindness in South Hampshire sheep: a neuronal ceroidlipofuscinosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

Sheep and other animals with ceroid-lipofuscinoses: their relevance to Batten disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

Comparative biology of the neuronal ceroid-lipofuscinoses (NCL): an overview.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

The ovine model of neuronal ceroid lipofuscinosis (NCL): its contribution to understanding the pathogenesis of Batten disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

The expression of tripeptidyl peptidase I in various tissues of rats and mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

Involvement of two different cell death pathways in retinal atrophy of cathepsin D-deficient mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

Microglia: a sensor for pathological events in the CNS

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

Delayed classic and protracted phenotypes of compound heterozygous juvenile neuronal ceroid lipofuscinosis.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

Palmitoyl protein thioesterase (PPT) localizes into synaptosomes and synaptic vesicles in neurons: implications for infantile neuronal ceroid lipofuscinosis (INCL)

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

Genetic markers linked to neuronal ceroid lipofuscinosis in English setter dogs.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

CLN3 protein is targeted to neuronal synapses but excluded from synaptic vesicles: new clues to Batten disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

Morphological alterations in neocortical and cerebellar GABAergic neurons in a canine model of juvenile Batten disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

An early-onset congenic strain of the motor neuron degeneration (mnd) mouse.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

Mutations in the palmitoyl-protein thioesterase gene (PPT; CLN1) causing juvenile neuronal ceroid lipofuscinosis with granular osmiophilic deposits [published erratum appears in Hum Mol Genet 1998 Apr;7(4):765]

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

Targeted disruption of the Cln3 gene provides a mouse model for Batten disease. The Batten Mouse Model Consortium [corrected]

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

Neurodegenerative disease: the neuronal ceroid lipofuscinoses (Batten disease).

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

Changes in GABAergic neuron distribution in situ and in neuron cultures in ovine (OCL6) Batten disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

Studies of pH regulation by Btn1p, the yeast homolog of human Cln3p

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

Motifs within the CLN3 protein: modulation of cell growth rates and apoptosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

Expanded CAG repeats in exon 1 of the Huntington's disease gene stimulate dopamine-mediated striatal neuron autophagy and degeneration

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

CLN3 defines a novel antiapoptotic pathway operative in neurodegeneration and mediated by ceramide

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

Optic nerve degeneration in a murine model of juvenile ceroid lipofuscinosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

CLN5, a novel gene encoding a putative transmembrane protein mutated in Finnish variant late infantile neuronal ceroid lipofuscinosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

Retinal pathology and function in a Cln3 knockout mouse model of juvenile Neuronal Ceroid Lipofuscinosis (batten disease).

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

Association of mutations in a lysosomal protein with classical late-infantile neuronal ceroid lipofuscinosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

Microglia as neuroprotective, immunocompetent cells of the CNS.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

Disruption of ClC-3, a chloride channel expressed on synaptic vesicles, leads to a loss of the hippocampus

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

A case of juvenile lipidosis: electron microscopic, histochemical and biochemical studies

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

Ceroid lipofuscinosis in the Border Collie dog: Retinal lesions in an animal model of juvenile Batten disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

Mechanisms of cell death in the inherited retinal degenerations

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

A mutation in the ovine cathepsin D gene causes a congenital lysosomal storage disease with profound neurodegeneration

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

Congenital ovine neuronal ceroid lipofuscinosis--a cathepsin D deficiency with increased levels of the inactive enzyme.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

Mutations in the palmitoyl protein thioesterase gene causing infantile neuronal ceroid lipofuscinosis

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

Neuroaxonal dystrophy in neuronal storage disorders: evidence for major GABAergic neuron involvement.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

Biology of neuronal dysfunction in storage disorders

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

Pathogenesis of brain dysfunction in Batten disease.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

The gene mutated in variant late-infantile neuronal ceroid lipofuscinosis (CLN6) and in nclf mutant mice encodes a novel predicted transmembrane protein

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

The cellular pathology of neuronal ceroid-lipofuscinosis. A golgi-electronmicroscopic study.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

Pheno/genotypic correlations of neuronal ceroid lipofuscinoses

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

CLC-3 deficiency leads to phenotypes similar to human neuronal ceroid lipofuscinosis.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

Diversity in the mechanisms of neuronal cell death.

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

Adult neuronal ceroid lipofuscinosis with palmitoyl-protein thioesterase deficiency: First adult-onset patients of a childhood disease

21 January 2018

1 reference

https://api.crossref.org/works/10.1111%2FJ.1750-3639.2004.TB00502.X

10.1111/J.1750-3639.2004.TB00502.X

21 January 2018

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DOI

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9 August 2017

1 reference