Toll-like receptor (TLR) pathway defects

AP1: activating protein 1; BTK: Bruton tyrosine kinase; IKK: inhibitor of nuclear factor kappa B kinase; IKKi: I-kappa-B kinase i; IRAK4:interleukin 1 receptor-associated kinase; IRF: interferon regulatory factor; MAL: MyD88 adapter-like; MAPK: mitogen-activated protein kinase; MD2: myeloid differentiation factor 2; MyD88: myeloid differentiation primary response protein 88; NEMO: nuclear factor-kappa-B essential modulator; NFkB: nuclear factor kappa B; OPNi: osteopontin i; TAK1: transforming growth factor beta-activated protein kinase 1; RIP1: receptor-interacting protein kinase 1; TLR: Toll-like receptor; TBK1: tank-binding kinase 1; TRAF: TNF receptor-associated factor; TRAM: TRIF-related adapter molecule; TRIF: Toll/interleukin 1 receptor domain-containing adapter-inducing interferon beta; UNC93B: UNC93 homolog B.