(Q37109009)

English

Location and connectivity determine GABAergic interneuron survival in the brains of South Hampshire sheep with CLN6 neuronal ceroid lipofuscinosis

scientific article published on 25 June 2008

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10 January 2020

Location and connectivity determine GABAergic interneuron survival in the brains of South Hampshire sheep with CLN6 neuronal ceroid lipofuscinosis (English)

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10 January 2020

neuronal ceroid lipofuscinosis

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Jonathan D. Cooper

5

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10 January 2020

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David N Palmer

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10 January 2020

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Manfred J Oswald

1

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10 January 2020

Graham W Kay

3

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10 January 2020

Karen J Barwell

4

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10 January 2020

publication date

25 June 2008

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10 January 2020

Neurobiology of Disease

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10 January 2020

32

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10 January 2020

1

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10 January 2020

50-65

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10 January 2020

A new large animal model of CLN5 neuronal ceroid lipofuscinosis in Borderdale sheep is caused by a nucleotide substitution at a consensus splice site (c.571+1G>A) leading to excision of exon 3.

1 reference

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14 September 2017

The novel neuronal ceroid lipofuscinosis gene MFSD8 encodes a putative lysosomal transporter

1 reference

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14 September 2017

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

1 reference

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14 September 2017

Progress towards understanding disease mechanisms in small vertebrate models of neuronal ceroid lipofuscinosis.

1 reference

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14 September 2017

The neuronal ceroid-lipofuscinoses: from past to present

1 reference

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Astrocyte dysfunction in neurological disorders: a molecular perspective

1 reference

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14 September 2017

EEG in Creutzfeldt-Jakob disease

1 reference

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14 September 2017

Inhibitory coupling specifically generates emergent gamma oscillations in diverse cell types

1 reference

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14 September 2017

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

1 reference

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14 September 2017

Mice lacking Dlx1 show subtype-specific loss of interneurons, reduced inhibition and epilepsy

1 reference

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14 September 2017

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2647510

14 September 2017

The human brain mannose 6-phosphate glycoproteome: a complex mixture composed of multiple isoforms of many soluble lysosomal proteins

1 reference

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14 September 2017

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

1 reference

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14 September 2017

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2647510

14 September 2017

Plasticity in single neuron and circuit computations

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2647510

14 September 2017

Does inhibition balance excitation in neocortex?

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2647510

14 September 2017

Interneurons of the neocortical inhibitory system

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2647510

14 September 2017

CLN6, which is associated with a lysosomal storage disease, is an endoplasmic reticulum protein

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2647510

14 September 2017

The mouse ortholog of the neuronal ceroid lipofuscinosis CLN5 gene encodes a soluble lysosomal glycoprotein expressed in the developing brain

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2647510

14 September 2017

Lysine 43 is trimethylated in subunit C from bovine mitochondrial ATP synthase and in storage bodies associated with batten disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2647510

14 September 2017

Defective endoplasmic reticulum-resident membrane protein CLN6 affects lysosomal degradation of endocytosed arylsulfatase A

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2647510

14 September 2017

Selectivity and types of cell death in the neuronal ceroid lipofuscinoses

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2647510

14 September 2017

Brain-derived neurotrophic factor is required for the maintenance of cortical dendrites

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2647510

14 September 2017

Cortical interneuron fate determination: diverse sources for distinct subtypes?

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2647510

14 September 2017

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2647510

14 September 2017

"Dormant basket cell" hypothesis revisited: relative vulnerabilities of dentate gyrus mossy cells and inhibitory interneurons after hippocampal status epilepticus in the rat

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2647510

14 September 2017

The neuronal ceroid-lipofuscinoses

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2647510

14 September 2017

Interlaminar connections in the neocortex

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2647510

14 September 2017

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2647510

14 September 2017

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2647510

14 September 2017

Dynamic predictions: oscillations and synchrony in top-down processing

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2647510

14 September 2017

On the cellular and network bases of epileptic seizures.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2647510

14 September 2017

Brain-derived neurotrophic factor in the control human brain, and in Alzheimer's disease and Parkinson's disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2647510

14 September 2017

Neuronal synchrony: a versatile code for the definition of relations?

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2647510

14 September 2017

The origin and migration of cortical neurones: new vistas

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2647510

14 September 2017

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2647510

14 September 2017

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2647510

14 September 2017

Ovine neuronal ceroid lipofuscinosis: a large animal model syntenic with the human neuronal ceroid lipofuscinosis variant CLN6.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2647510

14 September 2017

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2647510

14 September 2017

Interneurons of the hippocampus

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2647510

14 September 2017

Synaptic activity and the construction of cortical circuits

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2647510

14 September 2017

Thalamocortical oscillations in the sleeping and aroused brain

1 reference

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14 September 2017

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2647510

14 September 2017

Calbindin D-28k and parvalbumin in the rat nervous system

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2647510

14 September 2017

Preface: Cerebral Cortex Has Come of Age

1 reference

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14 September 2017

Horizontal integration and cortical dynamics

1 reference

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14 September 2017

Two motifs target Batten disease protein CLN3 to lysosomes in transfected nonneuronal and neuronal cells

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2647510

29 September 2017

Characterization of Cln3p, the gene product responsible for juvenile neuronal ceroid lipofuscinosis, as a lysosomal integral membrane glycoprotein

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2647510

29 September 2017

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2647510

29 September 2017

Striatal interneurones: chemical, physiological and morphological characterization

1 reference

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29 September 2017

Pathogenesis of brain dysfunction in Batten disease.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2647510

29 September 2017

Neurotrophins and neuronal plasticity

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2647510

29 September 2017

Cathepsin D deficiency underlies congenital human neuronal ceroid-lipofuscinosis

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2647510

27 June 2018

An astrocytic basis of epilepsy

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2647510

27 June 2018

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

1 reference

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27 June 2018

Serial MRI findings in the Costa Rican variant of neuronal ceroid-lipofuscinosis

1 reference

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27 June 2018

Origin and molecular specification of striatal interneurons.

1 reference

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27 June 2018

Cortical degeneration in the absence of neurotrophin signaling: dendritic retraction and neuronal loss after removal of the receptor TrkB.

1 reference

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27 June 2018

Three distinct subpopulations of GABAergic neurons in rat frontal agranular cortex

1 reference

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27 June 2018

Ceroid-lipofuscinosis (Batten's disease): pathogenesis of blindness in the ovine model

1 reference

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27 June 2018

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

1 reference

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27 June 2018

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

1 reference

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27 June 2018

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

1 reference

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3 September 2018

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.

1 reference

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3 September 2018

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

1 reference

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3 September 2018

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

1 reference

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3 September 2018

Inhibitory postsynaptic potentials carry synchronized frequency information in active cortical networks.

1 reference

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3 September 2018

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

1 reference

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3 September 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2647510

3 September 2018

Localization of wild-type and mutant neuronal ceroid lipofuscinosis CLN8 proteins in non-neuronal and neuronal cells

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2647510

3 September 2018

Parvalbumin deficiency affects network properties resulting in increased susceptibility to epileptic seizures.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2647510

3 September 2018

Neuroradiological findings (MRS, MRI, SPECT) in infantile neuronal ceroid-lipofuscinosis (infantile CLN1) at different stages of the disease

1 reference

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3 September 2018

Balanced inhibition underlies tuning and sharpens spike timing in auditory cortex

1 reference

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3 September 2018

Immunocytochemical localization of calretinin in the superficial layers of the cat superior colliculus

1 reference

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3 September 2018

Selective GABAergic innervation of thalamic nuclei from zona incerta

1 reference

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3 September 2018

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

1 reference

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3 September 2018

Types of neurons, synaptic connections and chemical characteristics of cells immunoreactive for calbindin-D28K, parvalbumin and calretinin in the neocortex

1 reference

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3 September 2018

Distribution of parvalbumin-immunoreactive neurons in brain correlates with hippocampal and temporal cortical pathology in Creutzfeldt-Jakob disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2647510

3 September 2018

Variant late infantile neuronal ceroid-lipofuscinosis: pathology and biochemistry

1 reference

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3 September 2018

Distributed hierarchical processing in the primate cerebral cortex

1 reference

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3 September 2018

Different forms of synaptic plasticity in somatosensory and motor areas of the neocortex

1 reference

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20 October 2018

Ceroid-lipofuscinosis (Batten's disease). Sequential electrophysiologic and pathologic changes in the retina of the ovine model

1 reference

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12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Mitochondrial ATP synthase subunit c storage in the ceroid-lipofuscinoses (Batten disease)

1 reference

https://pubmed.ncbi.nlm.nih.gov/18634879

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Parvalbumin-immunoreactive cortical neurons in Creutzfeldt-Jakob disease

1 reference

https://pubmed.ncbi.nlm.nih.gov/18634879

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/18634879

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Connectivity of GABAergic calretinin-immunoreactive neurons in rat primary visual cortex

1 reference

https://pubmed.ncbi.nlm.nih.gov/18634879

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Proximally targeted GABAergic synapses and gap junctions synchronize cortical interneurons

1 reference

https://pubmed.ncbi.nlm.nih.gov/18634879

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/18634879

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

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

1 reference

https://pubmed.ncbi.nlm.nih.gov/18634879

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Identifiers

10.1016/J.NBD.2008.06.004

1 reference

Europe PubMed Central

PMC publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18634879%20AND%20SRC:MED&resulttype=core&format=json

10 January 2020

PMC publication ID

1 reference

Europe PubMed Central

PMC publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18634879%20AND%20SRC:MED&resulttype=core&format=json

10 January 2020

PubMed publication ID

1 reference

Europe PubMed Central

PMC publication ID

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:18634879%20AND%20SRC:MED&resulttype=core&format=json

10 January 2020

ResearchGate publication ID

0 references

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