Evidence for aberrant astrocyte hemichannel activity in Juvenile Neuronal Ceroid Lipofuscinosis (JNCL). (Q35149204)

6 August 2017

Evidence for aberrant astrocyte hemichannel activity in Juvenile Neuronal Ceroid Lipofuscinosis (JNCL). (English)

1 reference

juvenile neuronal ceroid lipofuscinosis

6 August 2017

0 references

0 references

Hideyuki Takeuchi

6

1 reference

ORCID Public Data File 2021

author name string

Maria Burkovetskaya

1

1 reference

6 August 2017

Nikolay Karpuk

2

1 reference

6 August 2017

Juan Xiong

3

1 reference

6 August 2017

Megan Bosch

4

1 reference

6 August 2017

Michael D Boska

5

1 reference

6 August 2017

Akio Suzumura

7

1 reference

6 August 2017

Tammy Kielian

8

1 reference

6 August 2017

language of work or name

English

0 references

publication date

15 April 2014

1 reference

6 August 2017

1 reference

6 August 2017

9

1 reference

6 August 2017

4

1 reference

6 August 2017

e95023

1 reference

Creative Commons Attribution 4.0 International

6 August 2017

start time

15 April 2014

1 reference

April 2022 Public Data File from Crossref

copyright status

copyrighted

0 references

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Classification and natural history of the neuronal ceroid lipofuscinoses

13 July 2018

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Lysosomal membrane permeability stimulates protein aggregate formation in neurons of a lysosomal disease

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Large-scale phenotyping of an accurate genetic mouse model of JNCL identifies novel early pathology outside the central nervous system

13 July 2018

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Age-dependent therapeutic effect of memantine in a mouse model of juvenile Batten disease

13 July 2018

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Neuroinflammation alters voltage-dependent conductance in striatal astrocytes

13 July 2018

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The role of gap junction channels during physiologic and pathologic conditions of the human central nervous system

13 July 2018

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Juvenile neuronal ceroid lipofuscinoses

13 July 2018

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Mutation of the parkinsonism gene ATP13A2 causes neuronal ceroid-lipofuscinosis

13 July 2018

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The role of attenuated astrocyte activation in infantile neuronal ceroid lipofuscinosis

13 July 2018

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Carbenoxolone blocks the light-evoked rise in intracellular calcium in isolated melanopsin ganglion cell photoreceptors

13 July 2018

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Connexin26 expression in brain parenchymal cells demonstrated by targeted connexin ablation in transgenic mice

13 July 2018

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Blockade of gap junction hemichannel suppresses disease progression in mouse models of amyotrophic lateral sclerosis and Alzheimer's disease

13 July 2018

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13 July 2018

Physiology of microglia

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A metabolomic comparison of mouse models of the Neuronal Ceroid Lipofuscinoses

13 July 2018

1 reference

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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

13 July 2018

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Hemichannels in the neurovascular unit and white matter under normal and inflamed conditions

13 July 2018

1 reference

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Neuroinflammation leads to region-dependent alterations in astrocyte gap junction communication and hemichannel activity

13 July 2018

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Temporary inhibition of AMPA receptors induces a prolonged improvement of motor performance in a mouse model of juvenile Batten disease

13 July 2018

1 reference

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pH-dependent localization of Btn1p in the yeast model for Batten disease

13 July 2018

1 reference

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Increased coupling and altered glutamate transport currents in astrocytes following kainic-acid-induced status epilepticus

13 July 2018

1 reference

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Pharmacological and genetic approaches to study connexin-mediated channels in glial cells of the central nervous system

13 July 2018

1 reference

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Connexin channels and phospholipids: association and modulation

13 July 2018

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Direct actions of carbenoxolone on synaptic transmission and neuronal membrane properties

13 July 2018

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Pannexin 1: the molecular substrate of astrocyte "hemichannels".

13 July 2018

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Cerebellar defects in a mouse model of juvenile neuronal ceroid lipofuscinosis.

13 July 2018

1 reference

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Connexins, pannexins, innexins: novel roles of "hemi-channels"

13 July 2018

1 reference

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Connexin and pannexin mediated cell-cell communication.

13 July 2018

1 reference

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Glial connexins and gap junctions in CNS inflammation and disease

13 July 2018

1 reference

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Neuronal pigmented autophagic vacuoles: lipofuscin, neuromelanin, and ceroid as macroautophagic responses during aging and disease

13 July 2018

1 reference

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Gene expression profiling in a mouse model of infantile neuronal ceroid lipofuscinosis reveals upregulation of immediate early genes and mediators of the inflammatory response

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3988164

Pannexins and gap junction protein diversity

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3988164

Juvenile neuronal ceroid-lipofuscinosis (Batten disease): a brief review and update

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3988164

Modulation of connexin expression and gap junction communication in astrocytes by the gram-positive bacterium S. aureus

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3988164

Functional connexin “hemichannels”: A critical appraisal

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3988164

Brain lipopigment accumulation in normal and pathological aging

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3988164

Possible involvement of gap junctions in the barrier function of tight junctions of brain and lung endothelial cells

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3988164

You say lipofuscin, we say ceroid: defining autofluorescent storage material

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3988164

Astrocyte-mediated control of cerebral blood flow

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3988164

Instrumentation for molecular imaging in preclinical research: Micro-PET and Micro-SPECT.

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3988164

High resolution 1H NMR-based metabolomics indicates a neurotransmitter cycling deficit in cerebral tissue from a mouse model of Batten disease

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3988164

Staphylococcus aureus-derived peptidoglycan induces Cx43 expression and functional gap junction intercellular communication in microglia

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3988164

Astrocytes, from brain glue to communication elements: the revolution continues

13 July 2018

1 reference

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Sulforhodamine 101 as a specific marker of astroglia in the neocortex in vivo

13 July 2018

1 reference

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Mice with Ppt1Deltaex4 mutation replicate the INCL phenotype and show an inflammation-associated loss of interneurons

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3988164

Membrane trafficking and mitochondrial abnormalities precede subunit c deposition in a cerebellar cell model of juvenile neuronal ceroid lipofuscinosis

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3988164

Mouse MRI: concepts and applications in physiology

13 July 2018

1 reference

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New roles for astrocytes: Gap junction hemichannels have something to communicate

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3988164

New roles for astrocytes (stars at last).

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3988164

Glutathione pathways in the brain.

13 July 2018

1 reference

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Evaluation of fluorescent dyes for measuring intracellular glutathione content in primary cultures of human neurons and neuroblastoma SH-SY5Y.

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3988164

Magnetic resonance spectroscopy in AD.

13 July 2018

1 reference

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Microglia at brain stab wounds express connexin 43 and in vitro form functional gap junctions after treatment with interferon-gamma and tumor necrosis factor-alpha

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3988164

GLIA: listening and talking to the synapse

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3988164

Glial modulation of neural excitability mediated by extracellular pH: a hypothesis revisited

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3988164

Astrocyte glutamate transport: review of properties, regulation, and physiological functions

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3988164

Evaluation of glutathione-sensitive fluorescent dyes in cortical culture.

13 July 2018

1 reference

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Spectrum of mutations in the Batten disease gene, CLN3

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3988164

Live astrocytes visualized by green fluorescent protein in transgenic mice.

13 July 2018

1 reference

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Localized proton magnetic resonance spectroscopy of cerebral metabolic disturbances in children with neuronal ceroid lipofuscinosis.

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3988164

N-acetylaspartate in neuropsychiatric disorders

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3988164

Role of subunit-9 of mitochondrial ATP synthase in Batten disease

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3988164

Isolation of a novel gene underlying Batten disease, CLN3. The International Batten Disease Consortium

13 July 2018

1 reference

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Ceroid-lipofuscinosis in Border Collie dogs

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3988164

Regional brain atrophy in mouse models of neuronal ceroid lipofuscinosis: a new rostrocaudal perspective

13 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3988164

Microglia in juvenile neuronal ceroid lipofuscinosis are primed toward a pro-inflammatory phenotype.

27 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3988164

Injection of neural progenitor cells attenuates decrease in level of connexin 43 in brain capillaries after cerebral ischemia

27 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3988164

Immune cells perturb axons and impair neuronal survival in a mouse model of infantile neuronal ceroid lipofuscinosis.

27 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3988164

Sudan Black B treatment reduces autofluorescence and improves resolution of in situ hybridization specific fluorescent signals of brain sections

27 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3988164

Astroglial metabolic networks sustain hippocampal synaptic transmission

27 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3988164

Developmental impairments of select neurotransmitter systems in brains of Cln3(Deltaex7/8) knock-in mice, an animal model of juvenile neuronal ceroid lipofuscinosis

27 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3988164

The impact of astrocytic gap junctional coupling on potassium buffering in the hippocampus.

27 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3988164

Fully automated shim mapping method for spectroscopic imaging of the mouse brain at 9.4 T.

27 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3988164

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

27 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3988164

Time-domain semi-parametric estimation based on a metabolite basis set.

27 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3988164

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

27 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3988164

Calcium transients in astrocyte endfeet cause cerebrovascular constrictions

27 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3988164

Time-domain quantitation of 1H short echo-time signals: background accommodation.

27 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3988164

High glucose alters connexin 43 expression and gap junction intercellular communication activity in retinal pericytes

27 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3988164

Carbenoxolone Blockade of Neuronal Network Activity in Culture is not Mediated by an Action on Gap Junctions

27 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3988164

alpha-synuclein immunopositive Parkinson's disease-related inclusion bodies in lower brain stem nuclei.

27 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3988164

Neuronal ceroid-lipofuscinosis in a domestic cat: clinical, morphological and immunohistochemical findings.

27 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3988164

Heptanol-induced decrease in cardiac gap junctional conductance is mediated by a decrease in the fluidity of membranous cholesterol-rich domains.

27 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3988164

27 September 2018

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12 December 2020

Cln3(Deltaex7/8) knock-in mice with the common JNCL mutation exhibit progressive neurologic disease that begins before birth

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

Amyloid β-induced death in neurons involves glial and neuronal hemichannels

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12 December 2020

Late-infantile Batten disease: purification of the subunit c of the mitochondrial ATP synthase from storage material

1 reference

12 December 2020

10.1371/JOURNAL.PONE.0095023

Identifiers

DOI

1 reference

6 August 2017

0 references

1 reference

6 August 2017

1 reference