Kay, Gaham W.; Palmer, David N.; Rezaie, Payam and Cooper, Jonathan D.
(2006).
 | This is the latest version of this eprint. |
| DOI (Digital Object Identifier) Link: | https://doi.org/10.1111/j.1750-3639.2006.00002.x |
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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.
| Item Type: | Journal Item |
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| ISSN: | 1015-6305 |
| Academic Unit/School: | Faculty of Science, Technology, Engineering and Mathematics (STEM) > Life, Health and Chemical Sciences Faculty of Science, Technology, Engineering and Mathematics (STEM) |
| Interdisciplinary Research Centre: | Biomedical Research Network (BRN) Health and Wellbeing PRA (Priority Research Area) |
| Item ID: | 2801 |
| Depositing User: | Payam Rezaie |
| Date Deposited: | 19 Jun 2006 |
| Last Modified: | 16 Aug 2017 15:52 |
| URI: | http://oro.open.ac.uk/id/eprint/2801 |
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Prenatal activation of non-neuronal cells within the developing CNS of sheep with neuronal ceroid lipofuscinosis (CLN6). (deposited 19 Jun 2006)
- Activation of non-neuronal cells within the prenatal developing brain of sheep with neuronal ceroid lipofuscinosis. (deposited 19 Jun 2006) [Currently Displayed]
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