[HTML][HTML] Mechanism of dysfunction of human variants of the IRAK4 kinase and a role for its kinase activity in interleukin-1 receptor signaling
S De, F Karim, E Kiessu, L Cushing, LL Lin… - Journal of Biological …, 2018 - ASBMB
Interleukin-1 receptor (IL1R)-associated kinase 4 (IRAK4) is a central regulator of innate
immune signaling, controlling IL1R and Toll-like receptor (TLR)-mediated responses and
containing both scaffolding and kinase activities. Humans deficient in IRAK4 activity have
autosomal recessive primary immune deficiency (PID). Here, we characterized the
molecular mechanism of dysfunction of two IRAK4 PID variants, G298D and the compound
variant R12C (R12C/R391H/T458I). Using these variants and the kinase-inactive D329A …
immune signaling, controlling IL1R and Toll-like receptor (TLR)-mediated responses and
containing both scaffolding and kinase activities. Humans deficient in IRAK4 activity have
autosomal recessive primary immune deficiency (PID). Here, we characterized the
molecular mechanism of dysfunction of two IRAK4 PID variants, G298D and the compound
variant R12C (R12C/R391H/T458I). Using these variants and the kinase-inactive D329A …
