IKBKG and immune system disorder: Infliximab has been trialled to target TNF-driven IBD-like inflammation seen in X-linked ectodermal dysplasia and immunodeficiency, an epithelial cell defect secondary to mutations in IKBKG/NEMO, in which defective NF-κB activation impairs immune response to circulating TNF [42], as well as Hermansky-Pudlak syndrome, a hyperinflammatory disorder involving defective cellular trafficking of lysosome-related organelles [43].