NLRP3 and nervous system disorder: However, an expanding body of evidence suggests that neuroinflammation is a pathogenetic machinery of utmost importance in many neurological disorders, and that TLR4/IL-1 dependent priming with activation of the NLRP3-inflammasome assembly followed by IL-1β/IL-18 maturation and pyroptosis are determinant in eliciting both neurocognitive decline and behavioral abnormalities in the Sanfilippo syndrome [75].