Purified recombinant fragment of human IRAK-4 expressed in E. Coli.

IRAK-4 Monoclonal Antibody detects endogenous levels of IRAK-4 protein.

IRAK4

Interleukin-1 receptor-associated kinase 4

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. [provided by RefSeq, Aug 2011],

Catalytic activity:ATP + a protein = ADP + a phosphoprotein.,cofactor:Magnesium.,Disease:Defects in IRAK4 are the cause of IRAK4 deficiency [MIM:607676]. IRAK4 deficiency causes extracellular pyogenic bacterial and fungal infections in otherwise healthy children.,Disease:Defects in IRAK4 are the cause of recurrent isolated invasive pneumococcal disease type 1 (IPD1) [MIM:610799]. Recurrent invasive pneumococcal disease (IPD) is defined as two episodes of IPD occurring at least 1 month apart, whether caused by the same or different serotypes or strains. Recurrent IPD occurs in at least 2% of patients in most series, making IPD the most important known risk factor for subsequent IPD.,Function:Required for the efficient recruitment of IRAK1 to the IL-1 receptor complex following IL-1 engagement, triggering intracellular signaling cascades leading to transcriptional up-regulation and mRNA stabilization. Phosphorylates IRAK1.,online information:IRAK4 mutation db,similarity:Belongs to the protein kinase superfamily. TKL Ser/Thr protein kinase family. Pelle subfamily.,similarity:Contains 1 death domain.,similarity:Contains 1 protein kinase domain.,subunit:IL-1 stimulation leads to the formation of a signaling complex which dissociates from the IL-1 receptor following the binding of PELI1. Interacts with IL1RL1.,

extracellular space,intracellular,nucleus,cytoplasm,cytosol,plasma membrane,endosome membrane,

Brain,Spleen,Uterus,