A murine model of variant late infantile ceroid lipofuscinosis recapitulates behavioral and pathological phenotypes of human disease (Q27303003)

1

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Helen Magee

2

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Andrew Wong

3

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Tarah Nelson

4

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Bettina Koch

5

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Jill M Weimer

Creative Commons Attribution 4.0 International

7

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language of work or name

English

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publication date

2013

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published in

PLOS One

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volume

8

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issue

11

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page(s)

e78694

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copyright license

start time

1 November 2013

1 reference

April 2022 Public Data File from Crossref

copyright status

copyrighted

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describes a project that uses

ImageJ

1 reference

Europe PubMed Central

11 June 2022

https://www.ebi.ac.uk/europepmc/webservices/rest/PMC3815212/fullTextXML

inferred from PubMed Central ID database lookup

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28 September 2017

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

28 September 2017

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31 May 2018

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Ceroid-lipofuscinosis (Batten's disease): pathogenesis and sequential neuropathological changes in the ovine model

31 May 2018

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An ultrastructural study on retinal neural and pigment epithelial cells in ovine neuronal ceroid-lipofuscinosis

31 May 2018

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The spectrum of Jansky-Bielschowsky disease.

31 May 2018

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31 May 2018

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Clinicopathological and molecular characterization of neuronal ceroid lipofuscinosis in the Portuguese population.

21 January 2018

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A missense mutation (c.184C>T) in ovine CLN6 causes neuronal ceroid lipofuscinosis in Merino sheep whereas affected South Hampshire sheep have reduced levels of CLN6 mRNA.

21 January 2018

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The origin of fluorescence in the neuronal ceroid lipofuscinoses (Batten disease) and neuron cultures from affected sheep for studies of neurodegeneration

21 January 2018

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Metabolomic investigation of CLN6 neuronal ceroid lipofuscinosis in affected South Hampshire sheep.

21 January 2018

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Glial activation spreads from specific cerebral foci and precedes neurodegeneration in presymptomatic ovine neuronal ceroid lipofuscinosis (CLN6).

21 January 2018

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Neuronal ceroid lipofuscinosis in Merino sheep.

21 January 2018

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Early remodeling in an inducible animal model of retinal degeneration.

21 January 2018

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Sensorimotor and cognitive deficits after transient middle cerebral artery occlusion in the mouse.

21 January 2018

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Pole test is a useful method for evaluating the mouse movement disorder caused by striatal dopamine depletion.

21 January 2018

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Memory impairment and hippocampus specific protein oxidation induced by ethanol intake and 3, 4-methylenedioxymethamphetamine (MDMA) in mice.

21 January 2018

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Regional and cellular neuropathology in the palmitoyl protein thioesterase-1 null mutant mouse model of infantile neuronal ceroid lipofuscinosis

21 January 2018

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Late onset neurodegeneration in the Cln3-/- mouse model of juvenile neuronal ceroid lipofuscinosis is preceded by low level glial activation.

21 January 2018

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Thalamocortical neuron loss and localized astrocytosis in the Cln3Deltaex7/8 knock-in mouse model of Batten disease.

21 January 2018

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Selective spatiotemporal patterns of glial activation and neuron loss in the sensory thalamocortical pathways of neuronal ceroid lipofuscinosis 8 mice

21 January 2018

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Distinct neuropathologic phenotypes after disrupting the chloride transport proteins ClC-6 or ClC-7/Ostm1.

21 January 2018

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Neuronal ceroid lipofuscinosis: clinical and morphologic findings in nine affected Polish Owczarek Nizinny (PON) dogs.

21 January 2018

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

21 January 2018

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Altered gene expression in the eye of a mouse model for batten disease.

21 January 2018

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Increased dendritic spine densities on cortical projection neurons in autism spectrum disorders

21 January 2018

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Activation of non-neuronal cells within the prenatal developing brain of sheep with neuronal ceroid lipofuscinosis.

21 January 2018

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Equine neuronal ceroid lipofuscinosis.

21 January 2018

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Variant late infantile neuronal ceroid-lipofuscinosis: pathology and biochemistry.

28 November 2018

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Same gene, surprising difference: adult neuronal ceroid lipofuscinosis linked to CLN6, mutated in variant late-infantile form

28 November 2018

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12 December 2020

Synaptic Changes in the Thalamocortical System of Cathepsin D-Deficient Mice

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12 December 2020

Variant late infantile ceroid lipofuscinoses associated with novel mutations in CLN6

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12 December 2020

The neuronal ceroid-lipofuscinoses

1 reference

12 December 2020

Hippocampal pathology in the human neuronal ceroid-lipofuscinoses: distinct patterns of storage deposition, neurodegeneration and glial activation

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12 December 2020

Neuronal ceroid lipofuscinosis in Australian Merino sheep: a new animal model

1 reference

12 December 2020

The neuronal ceroid lipofuscinoses: the same, but different?

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12 December 2020

Retina in various animal models of neuronal ceroid-lipofuscinosis

1 reference

12 December 2020

Different patterns of hydrophobic protein storage in different forms of neuronal ceroid lipofuscinosis (NCL, Batten disease)

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12 December 2020

10.1371/JOURNAL.PONE.0078694

Identifiers

DOI

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ADS bibcode

2013PLoSO...878694M

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Fatcat ID

release_ymfauhhhxzh5hjst5rawj6yyja

1 reference

Fatcat

https://api.fatcat.wiki/v0/release/ymfauhhhxzh5hjst5rawj6yyja

24 November 2022