Given that the endosomal maturation inhibitor hydroxychloroquine inhibits TLR9 activation and is a common treatment for SjS [27], we injected NOD and BALB/c mice with ODN2088, which dramatically increased salivation in NOD mice (p = 0.0018) (Fig. 2D). Here, TLR9 is linked to Schwartz-Jampel syndrome.