Article
Conditional Niemann-Pick C mice demonstrate cell autonomous Purkinje cell neurodegeneration
10 Dec 2009
Abstract excerpt
Pathways regulating neuronal vulnerability are poorly understood, yet are central to identifying therapeutic targets for degenerative neurological diseases. Here, we characterize mechanisms underlying neurodegeneration in Niemann-Pick type C (NPC) disease, a lysosomal storage disorder characterized by impaired cholesterol trafficking. To date, the relative contributions of neuronal and glial defects to neuron...
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