In GD, inherited deficiency of the acidic β-glucosidase enzyme results in progressive lysosomal accumulation of βGL1 and LGL1. Upon recognition of CD1d-βGL1 or CD1d-LGL1 complexes on the surface of B cells and myeloid cells, type II NKT cells release a plethora of cytokines, including interleukin-2 (IL-2), interferon-γ (IFN-γ), IL-17, and IL-22. Crosstalk with myeloid cells results in their activation and secretion of inflammatory cytokines, such as MIP1-β, IL-6, and IL-8. Crosstalk with B cells leads to their activation, germinal center reaction, and immunoglobulin secretion.