(Q35785612)

10 August 2017

title

Successive neuron loss in the thalamus and cortex in a mouse model of infantile neuronal ceroid lipofuscinosis (English)

1 reference

Infantile neuronal ceroid lipofuscinosis

10 August 2017

main subject

0 references

0 references

Catherine Kielar

1 reference

10 August 2017

Lucy Maddox

1 reference

10 August 2017

Ellen Bible

1 reference

10 August 2017

Charlie C Pontikis

1 reference

10 August 2017

Shannon L Macauley

1 reference

10 August 2017

Megan A Griffey

1 reference

10 August 2017

Michael Wong

1 reference

10 August 2017

Mark S Sands

1 reference

10 August 2017

Jonathan D Cooper

1 reference

10 August 2017

language of work or name

1 reference

12 October 2006

1 reference

10 August 2017

published in

Neurobiology of Disease

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10 August 2017

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10 August 2017

1 reference

10 August 2017

page(s)

150-162

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Ocular phenotype in a mouse gene knockout model for infantile neuronal ceroid lipofuscinosis

10 August 2017

cites work

1 reference

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Endoplasmic reticulum stress-induced caspase-4 activation mediates apoptosis and neurodegeneration in INCL.

12 July 2018

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12 July 2018

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Visual deficits in a mouse model of Batten disease are the result of optic nerve degeneration and loss of dorsal lateral geniculate thalamic neurons

12 July 2018

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Palmitoyl-protein thioesterase-1 deficiency mediates the activation of the unfolded protein response and neuronal apoptosis in INCL

12 July 2018

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Progressively reduced synaptic vesicle pool size in cultured neurons derived from neuronal ceroid lipofuscinosis-1 knockout mice

12 July 2018

1 reference

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Signaling between glia and neurons: focus on synaptic plasticity

12 July 2018

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12 July 2018

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12 July 2018

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Correlations between genotype, ultrastructural morphology and clinical phenotype in the neuronal ceroid lipofuscinoses

12 July 2018

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12 July 2018

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The reticular nucleus revisited: intrinsic and network properties of a thalamic pacemaker

12 July 2018

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A mouse model of classical late-infantile neuronal ceroid lipofuscinosis based on targeted disruption of the CLN2 gene results in a loss of tripeptidyl-peptidase I activity and progressive neurodegeneration

12 July 2018

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

12 July 2018

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12 July 2018

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The effects of lysosomotropic agents on normal and INCL cells provide further evidence for the lysosomal nature of palmitoyl-protein thioesterase function

12 July 2018

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Centromedian-thalamic and hippocampal electrical stimulation for the control of intractable epileptic seizures

12 July 2018

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

12 July 2018

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Positional candidate gene cloning of CLN1.

12 July 2018

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Microglial response to brain injury: a brief synopsis

12 July 2018

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Apparent loss and hypertrophy of interneurons in a mouse model of neuronal ceroid lipofuscinosis: evidence for partial response to insulin-like growth factor-1 treatment

12 July 2018

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

12 July 2018

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Adult ceroid-lipofuscinosis: diagnostic value of biopsies and of neurophysiological investigations

12 July 2018

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

12 July 2018

1 reference

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Infantile type of so-called neuronal ceroid-lipofuscinosis. 1. A clinical study of 15 patients

12 July 2018

1 reference

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

12 July 2018

1 reference

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The efficiency of systematic sampling in stereology and its prediction.

12 July 2018

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Evoked Potentials in Neuronal Ceroid Lipofuscinosis

12 July 2018

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

12 July 2018

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CNS-directed AAV2-mediated gene therapy ameliorates functional deficits in a murine model of infantile neuronal ceroid lipofuscinosis.

25 September 2018

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

25 September 2018

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

25 September 2018

1 reference

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AAV2-mediated ocular gene therapy for infantile neuronal ceroid lipofuscinosis.

25 September 2018

1 reference

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

25 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1866219

Adeno-associated virus 2-mediated gene therapy decreases autofluorescent storage material and increases brain mass in a murine model of infantile neuronal ceroid lipofuscinosis

25 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1866219

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

25 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1866219

Somatosensory evoked magnetic fields from primary sensorimotor cortex in juvenile neuronal ceroid lipofuscinosis.

25 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1866219

MRI of neuronal ceroid lipofuscinosis. II. Postmortem MRI and histopathological study of the brain in 16 cases of neuronal ceroid lipofuscinosis of juvenile or late infantile type

25 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=1866219

EEG and evoked potentials in infantile neuronal ceroid-lipofuscinosis

25 September 2018

1 reference

PubMed

https://pubmed.ncbi.nlm.nih.gov/17046272

inferred from PubMed ID database lookup

12 December 2020

based on heuristic

Neuroglial activation repertoire in the injured brain: graded response, molecular mechanisms and cues to physiological function

1 reference

PubMed

https://pubmed.ncbi.nlm.nih.gov/17046272

inferred from PubMed ID database lookup

12 December 2020

based on heuristic

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

1 reference

PubMed

https://pubmed.ncbi.nlm.nih.gov/17046272

inferred from PubMed ID database lookup

12 December 2020

based on heuristic

Identifiers

DOI

10.1016/J.NBD.2006.09.001

1 reference

10 August 2017

1 reference

10 August 2017

1 reference