Discover Community Create You

Loading publication...

Noodle always counts identifier-free feature events for service operation. With your permission, Google Analytics can also estimate audience and engagement. Search text, publication identifiers, cookies, IP addresses and browser fingerprints are never included in Noodle's first-party event counters.

Privacy details

Article

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

Neurobiology of disease - 1 Oct 2008

Oswald Manfred J, Palmer David N, Kay Graham W, Barwell Karen J, Cooper Jonathan D

Abstract excerpt

The neuronal ceroid lipofuscinoses (NCLs, Batten disease) are fatal inherited neurodegenerative diseases. Sheep affected with the CLN6 form provide a valuable model to investigate underlying disease mechanisms from preclinical stages. Excitatory neuron loss in these sheep is markedly regional, localized early reactive changes accurately predicting neuron loss and subsequent symptom development. This investigation...

Read the complete abstract on PubMed

Topics

Share this publication in a Topic to start or enrich a Post.

Share to Brain
Share to Cell Survival

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to gamma-Aminobutyric Acid

Share to Interneurons

Share to Neural Pathways

Share to Neuronal Ceroid-Lipofuscinoses

Share to New Zealand

Share to Sheep, Domestic

Source

Open in PubMed Open full text

Identifiers

PMID: 18634879
DOI: 10.1016/j.nbd.2008.06.004
PMCID: PMC2647510

Genes

CLN6

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

Semantic proximity does not establish scientific evidence.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

Location and connectivity determine GABAergic interneuron survival in the brains of South Hampshire sheep with CLN6 neuronal ceroid lipofuscinosis.PMID 18634879Read ↗

Characterization of neuropathology in ovine CLN5 and CLN6 neuronal ceroid lipofuscinoses (Batten disease).Semantic proximity Read ↗

Rapid and Progressive Regional Brain Atrophy in CLN6 Batten Disease Affected Sheep Measured with Longitudinal Magnetic Resonance Imaging.Semantic proximity Read ↗

The neuronal ceroid lipofuscinoses: the same, but different?Semantic proximity Read ↗

A murine model of variant late infantile ceroid lipofuscinosis recapitulates behavioral and pathological phenotypes of human disease.Semantic proximity Read ↗

Progress towards understanding the neurobiology of Batten disease or neuronal ceroid lipofuscinosisSemantic proximity Read ↗

Increased zinc and manganese in parallel with neurodegeneration, synaptic protein changes and activation of Akt/GSK3 signaling in ovine CLN6 neuronal ceroid lipofuscinosis.Semantic proximity Read ↗

Prodromal pathogenesis of CLN7 Batten Disease revealed by multimodal biomarkers in macaquesSemantic proximity Read ↗

Molecular correlates of axonal and synaptic pathology in mouse models of Batten diseaseSemantic proximity Read ↗

Progress towards understanding the neurobiology of Batten disease or neuronal ceroid lipofuscinosis.Semantic proximity Read ↗

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

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.Semantic proximity Read ↗

Neuronal ceroid lipofuscinosis (NCL) is caused by the entire deletion of CLN8 in the Alpenländische Dachsbracke dog.Semantic proximity Read ↗

⌁Select a neighboring publication to make it the new centre.