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Juan Xiong and Tammy Kielian Microglia in juvenile neuronal ceroid lipofuscinosis are primed toward a pro-inflammatory phenotype Journal of Neurochemistry 127

Version of Record online: 22 AUG 2013 | DOI: 10.1111/jnc.12385

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Juvenile neuronal ceroid lipofuscinosis (JNCL) is a lysosomal storage disease caused by an autosomal recessive mutation in CLN3. Regions of microglial activation precede and predict areas of neuronal loss in JNCL; however, the functional role of activated microglia remains to be defined. In this report, primary microglia from CLN3Δex7/8 mutant mice over-produced numerous inflammatory cytokines in response to stimuli that are present in the JNCL brain, whereas wild-type microglia were relatively non-responsive. In addition, activated microglia induced significant cell death in CLN3Δex7/8 but not wild-type neurons, demonstrating that intrinsically diseased CLN3Δex7/8 neurons are less equipped to withstand cytotoxic insults. Collectively, aberrant microglial activation may contribute to the pathological chain of events leading to neurodegeneration during later stages of JNCL.

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