Article
Mechanism of dysfunction of human variants of the IRAK4 kinase and a role for its kinase activity in interleukin-1 receptor signaling.
The Journal of biological chemistry - 28 Sept 2018
De Saurav, Karim Fawziya, Kiessu Ezechielle, Cushing Leah, Lin Lih-Ling, Ghandil Pegah, Hoarau Cyrille, Casanova Jean-Laurent, Puel Anne, Rao Vikram R
Abstract excerpt
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...
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