Niemann-Pick C1 Mice, a Model of “Juvenile Alzheimer's Disease”, with Normal Gene Expression in Neurons and Fibrillary Astrocytes Show Long Term Survival and Delayed Neurodegeneration

Abstract excerpt

Niemann-Pick C1 (NPC) disease, also known as "juvenile Alzheimer's disease", is a disease in which alterations in intracellular cholesterol trafficking occur. The contribution of various CNS cell types to the neurodegeneration has been of much interest. We have previously shown that expression of the normal gene only in fibrillary astrocytes could extend survival of Npc1-/- mice over 3-fold (Zhang et al., 2008...

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