Location and connectivity determine GABAergic interneuron survival in the brains of South Hampshire sheep with CLN6 neuronal ceroid lipofuscinosis (Q37109009)

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scientific article published on 25 June 2008

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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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scholarly article

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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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based on heuristic

inferred from title

Jonathan D. Cooper

5

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10 January 2020

2

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David N Palmer

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10 January 2020

author name string

Manfred J Oswald

1

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10 January 2020

Graham W Kay

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10 January 2020

Karen J Barwell

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publication date

25 June 2008

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Neurobiology of Disease

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50-65

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1

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

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

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

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The neuronal ceroid-lipofuscinoses: from past to present

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Astrocyte dysfunction in neurological disorders: a molecular perspective

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

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

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

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Interneurons of the neocortical inhibitory system

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

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

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

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

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Selectivity and types of cell death in the neuronal ceroid lipofuscinoses

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

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

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The neuronal ceroid-lipofuscinoses

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Interlaminar connections in the neocortex

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

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

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

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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

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14 September 2017

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

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

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Interneurons of the hippocampus

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Cathepsin D deficiency underlies congenital human neuronal ceroid-lipofuscinosis

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27 June 2018

An astrocytic basis of epilepsy

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

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

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Three distinct subpopulations of GABAergic neurons in rat frontal agranular cortex.

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27 June 2018

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

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27 June 2018

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

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27 June 2018

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27 June 2018

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

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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.

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

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3 September 2018

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

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3 September 2018

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

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3 September 2018

Selective GABAergic innervation of thalamic nuclei from zona incerta

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3 September 2018

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

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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

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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.

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3 September 2018

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

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Distributed hierarchical processing in the primate cerebral cortex

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

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20 October 2018

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

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12 December 2020

based on heuristic

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

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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

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

10 January 2020

1 reference

Europe PubMed Central

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

10 January 2020

1 reference

Europe PubMed Central

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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