Selective spatiotemporal patterns of glial activation and neuron loss in the sensory thalamocortical pathways of neuronal ceroid lipofuscinosis 8 mice
Abstract
The neuronal ceroid lipofuscinoses constitute the most common group of childhood neurodegenerative disorders. These devastating disorders still remain without effective treatment. The use of animal models has provided significant information about NCL pathogenesis, highlighting early glial activation and neuron loss in specific brain regions of affected animals. Here, we have characterized the timing and regional-specificity of the pathological events of CLN8 disease utilizing the Cln8 deficient mouse model, Cln8(mnd). We have studied the progression of neuron loss, astrocytosis and microglial activation from early to moderately symptomatic (1, 3 and 5 months) and late symptomatic (8 months) mice. In Cln8 deficiency, the somatosensory pathway comprising the thalamic ventral posterior nucleus (VPM/VPL) and the primary somatosensory cortex (S1BF) was found to be the most affected relay system. Scattered microglia that appeared partially activated were already present at 3 months of age, followed by astrocytosis and the loss of thalamic relay neurons at 5 months of age, with all these phenotypes and glial activation becoming more pronounced with disease progression. Reactive changes followed a similar pattern in the corresponding ...Continue Reading
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