Early glial activation, synaptic changes and axonal pathology in the thalamocortical system of Niemann-Pick type C1 mice

Article ID	Journal	Published Year	Pages	File Type
6022699	Neurobiology of Disease	2012	15 Pages	PDF

Abstract

âº Staging of neuropathology defined in Npc1â/â mouse model of Niemann-pick type C. âº The relationship between atrophy, neuron loss and glial activation are explored. âº Pathology was most pronounced in interconnecting sensory thalamocortical pathways. âº Glial activation precedes the onset of neuron loss. âº Pre-synaptic markers are rearranged and white matter is atrophied.

Keywords

GFAP CPU LSDs Axonal spheroids SNR VAMP2 S1BF MGN NPC SNAP-25 Au1 LGNd Astrocytes lysosomal storage disorders Thalamus Neurodegeneration lent Cortex primary visual cortex primary motor cortex primary auditory cortex lateral entorhinal cortex Microglia neuropathology Niemann–Pick type C Reticular nucleus ventral posterior nucleus dorsal lateral geniculate nucleus medial geniculate nucleus Synapse Caudate putamen Glial fibrillary acidic protein synaptosomal-associated protein of 25 kDa