Inanimal models of JNCL, characterized by mutations of CLN3, primary microglia werefound to be in a proinflammatory state (Xiong and Kielian, 2013), and microglialand astrocytic activation were shown to precede neural cell death (Pontikis et al., 2004). The gene discussed is CLN3; the disease is juvenile neuronal ceroid lipofuscinosis.