ArticleBrain pathology (Zurich, Switzerland)2006
Activation of non-neuronal cells within the prenatal developing brain of sheep with neuronal ceroid lipofuscinosis.
Article in Brain pathology (Zurich, Switzerland), 2006. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 22 papers.
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Who cites it
22 citing papers in PubMed, 55 citations in OpenAlex.
- Neuronal ceroid lipofuscinosis: underlying mechanisms and emerging therapeutic targets.Nature reviews. Neurology · 2025Review
- Article
- Article
- Evaluation of Disease Lesions in the Developing Canine MPS IIIA Brain.JIMD reports · 2019Article
- in vivo localization of the neuronal ceroid lipofuscinosis proteins, CLN3 and CLN7, at endogenous expression levels.Neurobiology of disease · 2017Article
- Moving towards effective therapeutic strategies for Neuronal Ceroid Lipofuscinosis.Orphanet journal of rare diseases · 2016Review
- Neuron-astrocyte interactions in neurodegenerative diseases: Role of neuroinflammation.Clinical & experimental neuroimmunology · 2015Article
- Deregulation of subcellular biometal homeostasis through loss of the metal transporter, Zip7, in a childhood neurodegenerative disorder.Acta neuropathologica communications · 2014Article
- Chronic oral administration of minocycline to sheep with ovine CLN6 neuronal ceroid lipofuscinosis maintains pharmacological concentrations in the brain but does not suppress neuroinflammation or disease progression.Journal of neuroinflammation · 2013Article
- Article
- A murine model of variant late infantile ceroid lipofuscinosis recapitulates behavioral and pathological phenotypes of human disease.PloS one · 2013Article
- Disruption of the autophagy-lysosome pathway is involved in neuropathology of the nclf mouse model of neuronal ceroid lipofuscinosis.PloS one · 2012Article
- Therapeutic approaches to the challenge of neuronal ceroid lipofuscinoses.Current pharmaceutical biotechnology · 2011Review
- Pathophysiology of neuropathic lysosomal storage disorders.Journal of inherited metabolic disease · 2010Review
- [NCL in animal models].Der Ophthalmologe : Zeitschrift der Deutschen Ophthalmologischen Gesellschaft · 2010Article
- Neuropeptide changes and neuroactive amino acids in CSF from humans and sheep with neuronal ceroid lipofuscinoses (NCLs, Batten disease).Neurochemistry international · 2009Article
- Molecular correlates of axonal and synaptic pathology in mouse models of Batten disease.Human molecular genetics · 2009Article
- Location and connectivity determine GABAergic interneuron survival in the brains of South Hampshire sheep with CLN6 neuronal ceroid lipofuscinosis.Neurobiology of disease · 2008Article
- 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.Neurobiology of disease · 2008Article
- Enzyme, cell and gene-based therapies for metachromatic leukodystrophy.Journal of inherited metabolic disease · 2007Review
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Authors and funding
4 authors at 1 institution in 1 country.
Funding
Abstract
The neuronal ceroid lipofuscinoses (NCLs, Batten disease) are fatal inherited lysosomal storage diseases of children characterized by increasing blindness, seizures and profound neurodegeneration but the mechanisms leading to these pathological changes remain unclear. Sheep with a CLN6 form that have a human-like brain and disease progression are invaluable for studying pathogenesis. A study of preclinical pathology in these sheep revealed localized glial activation at only 12 days of age, particularly in cortical regions that subsequently degenerate. This has been extended by examining fetal tissue from 60 days of gestation onwards. A striking feature was the presence of reactive astrocytes and the hypertrophy and proliferation of perivascular cells noted within the developing white matter of the cerebral cortex 40 days before birth. Astrocytic activation was evident within the cortical gray matter 20 days before birth, and was confined to the superficial laminae 12 days after birth. Clusters of activated microglia were detected in upper neocortical gray matter laminae shortly after birth. Neuronal development in affected sheep was undisturbed at these early ages. This prenatal activation of non-neuronal cells within the affected brain indicates the onset of pathogenesis during brain development and that an ordered sequence of glial activation precedes neurodegeneration.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.