Specific NPC1 loss in microglia of the mouse brain leads to neuroinflammation and results in synaptic loss

Ziel/Aim Microglial activation is one hallmark in the pathology of the lipid storage disease Niemann-Pick type C. It remains unclear if microgliosis is caused by lysosomal storage or as a reactive upregulation to neuronal cell death. Hence, we created a knock-out mouse model with a specific loss of NPC1 in microglia and characterized it by imaging biomarkers for inflammation and synaptic function.