Myeloid differentiation primary response protein MyD88
Function
Toll様受容体やIL-1受容体によるのシグナル経路に自然免疫反応の経路にアダプタータンパクとして含まれる。IRAK1, IRAK2, IRF7およびTRAF6を介してNFκBの活性化をもたらし、サイトカインの分泌、炎症反応をもたらす。IL-8の翻訳レベルの増加、IL-18によるシグナル伝達経路に含まれる。
IRF-1を活性化し核内への移行を促し、IFN-beta、iNOS、IL12A遺伝子の発現を誘導する。
Adapter protein involved in the Toll-like receptor and IL-1 receptor signaling pathway in the innate immune response. Acts via IRAK1, IRAK2, IRF7 and TRAF6, leading to NF-kappa-B activation, cytokine secretion and the inflammatory response. Increases IL-8 transcription. Involved in IL-18-mediated signaling pathway. Activates IRF1 resulting in its rapid migration into the nucleus to mediate an efficient induction of IFN-beta, NOS2/INOS, and IL12A genes By similarity. Ref.2 Ref.8 Ref.11 Ref.13
Subunit structure
ホモ二量体、TIRドメインをもつTIRAP. Binds to TLR2, TLR4, IRAK1, IRAK2 and IRAK4とヘテロ二量体を作る。
Homodimer. Also forms heterodimers with TIRAP. Binds to TLR2, TLR4, IRAK1, IRAK2 and IRAK4 via their respective TIR domains. Interacts with IL18R1 By similarity. Interacts with BMX, IL1RL1 and IRF7. Interacts with LRRFIP1 and LRRFIP2; this interaction positively regulates Toll-like receptor (TLR) signaling in response to agonist. Interacts with FLII. LRRFIP1 and LRRFIP2 compete with FLII for MYD88-binding. Interacts with IRF1 By similarity. Ref.8 Ref.9 Ref.10 Ref.11 Ref.12 Ref.13
Subcellular location
Cytoplasm Ref.8 Ref.9.
Tissue specificity
Ubiquitous. Ref.1
Domain
The intermediate domain (ID) is required for the phosphorylation and activation of IRAK By similarity.
Involvement in disease
Defects in MYD88 are the cause of MYD88 deficiency (MYD88D) [MIM:612260]; also known as recurrent pyogenic bacterial infections due to MYD88 deficiency. Patients suffer from autosomal recessive, life-threatening, often recurrent pyogenic bacterial infections, including invasive pneumococcal disease, and die between 1 and 11 months of age. Surviving patients are otherwise healthy, with normal resistance to other microbes, and their clinical status improved with age. Ref.13 Ref.15
Sequence similarities
Contains 1 death domain.
Contains 1 TIR domain.
