IRAK4 Recombinant Monoclonal Antibody

Uniprot Id

Q9NWZ3

Target Species

Human

Target Name

IRAK4

Target Full Name

Interleukin-1 receptor-associated kinase 4

Target Function

Serine/threonine-protein kinase that plays a critical role in initiating innate immune response against foreign pathogens. Involved in Toll-like receptor (TLR) and IL-1R signaling pathways. Is rapidly recruited by MYD88 to the receptor-signaling complex upon TLR activation to form the Myddosome together with IRAK2. Phosphorylates initially IRAK1, thus stimulating the kinase activity and intensive autophosphorylation of IRAK1. Phosphorylates E3 ubiquitin ligases Pellino proteins (PELI1, PELI2 and PELI3) to promote pellino-mediated polyubiquitination of IRAK1. Then, the ubiquitin-binding domain of IKBKG/NEMO binds to polyubiquitinated IRAK1 bringing together the IRAK1-MAP3K7/TAK1-TRAF6 complex and the NEMO-IKKA-IKKB complex. In turn, MAP3K7/TAK1 activates IKKs (CHUK/IKKA and IKBKB/IKKB) leading to NF-kappa-B nuclear translocation and activation. Alternatively, phosphorylates TIRAP to promote its ubiquitination and subsequent degradation. Phosphorylates NCF1 and regulates NADPH oxidase activation after LPS stimulation suggesting a similar mechanism during microbial infections.

Target Involvement

Recurrent isolated invasive pneumococcal disease 1 (IPD1); IRAK4 deficiency (IRAK4D)

Target Subcellular Location

Cytoplasm.

Target Protein Families

Protein kinase superfamily, TKL Ser/Thr protein kinase family, Pelle subfamily

Target Synonyms

IL-1 receptor-associated kinase 4; Interleukin 1 receptor associated kinase 4 mutant form 1; Interleukin-1 receptor-associated kinase 4; Interleukin1 receptor associated kinase 4; IPD1; IRAK 4; IRAK-4; IRAK4; IRAK4 mutated form 1; IRAK4_HUMAN; LOC 51135; NY REN 64; NY REN 64 antigen; NY-REN-64; REN64; Renal carcinoma antigen NY-REN-64

Target Background

This gene encodes a kinase that activates NF-kappaB in both the Toll-like receptor (TLR) and T-cell receptor (TCR) signaling pathways. The protein is essential for most innate immune responses. Mutations in this gene result in IRAK4 deficiency and recurrent invasive pneumococcal disease. Multiple transcript variants encoding different isoforms have been found for this gene.