Figure 1. Molecular pro-inflammatory pathway activated in the central nervous system in patients with Mucopolysaccharidosis type III. The activation in glial cells occurs in a MyD88-independent pathway, with translocation of the TRIF-TRAM complex and subsequent activation of TRAF6. Released cytokines and pro-inflammatory products activate, via TLR4 receptors, a pro-inflammatory pathway MyD88-dependent in neurons, with translocation of IRAK4-IRAK1 and subsequent activation of TRAF6. The events of TRAF6 activation resulting in two directions: i) release of NF-kB to the nucleus and expression of interleukines, as well TNF-alpha resulting in a positive feedback for pro-inflammatory activation via TNRR1 receptors; ii) MAPK signaling pathway, resulting in secondary tau-phosphorylation and release AP-1 to the translocation of AP-1 to the nucleus, resulting in expression of MIP-1α inducing pro-inflammatory citokines