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. 2020 Aug 18;64(3):565–578. doi: 10.1042/EBC20190090

Figure 3. Simplified model of immune activation by (lyso)-GSL in LSD.

Figure 3

1. Direct recognition of lyso-GSL by toll-like receptor 4 (TLR4). 2. CD1d-restricted lyso-GSL presentation and activation of type II iNKT cells. 3. iNKT cell-mediated activation of B cells and plasma cells. 4. Production of anti-(lyso)-GSL autoantibodies by plasma cells. 5. Continuous antibody production may result in genetic instability driving cancer cell formation. 6. Glucosylceramide (GC)-immune complexes (IC) interact with Fcγ-R inducing C5a (part of complement system), which by binding to C5aR1 triggers induction of more GlcCer (and connected GlcSph). APC, antigen presenting cell.