Selective neurodegeneration, without neurofibrillary tangles, in a mouse model of Niemann-Pick C disease

The BALB/c mouse model of Niemann‐Pick type C (NPC) disease exhibits neuropathological similarities to the human condition. There is an age‐related cerebral atrophy, demyelination of the corpus callosum, and degeneration of cerebellar Purkinje cells in the NPC mouse. In human NPC, many cortical and...

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Veröffentlicht in:	Journal of comparative neurology (1911) 2001-05, Vol.433 (3), p.415-425
Hauptverfasser:	German, Dwight C., Quintero, E. Matthew, Liang, Chang-Lin, Ng, Benton, Punia, Surender, Xie, Chonglun, Dietschy, John M.
Format:	Artikel
Sprache:	eng
Schlagworte:	Animals Choline O-Acetyltransferase - metabolism cholinergic neurons corpus callosum Disease Models, Animal Female glial cells Histocytochemistry Male Mice Mice, Inbred BALB C - genetics Microscopy, Electron Mutation Nerve Degeneration - pathology Neurofibrillary Tangles - pathology Neurofibrillary Tangles - ultrastructure Neuroglia - pathology Neurons - enzymology Neurons - pathology Niemann-Pick Diseases - genetics Niemann-Pick Diseases - pathology prefrontal cortex Purkinje cells stereology thalamus
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