IL1R1

Domain

The TIR domain mediates NAD(+) hydrolase (NADase) activity. Self-association of TIR domains is required for NADase activity.

Function

Receptor for IL1A, IL1B and IL1RN (PubMed:2950091, PubMed:37315560). After binding to interleukin-1 associates with the coreceptor IL1RAP to form the high affinity interleukin-1 receptor complex which mediates interleukin-1-dependent activation of NF-kappa-B, MAPK and other pathways. Signaling involves the recruitment of adapter molecules such as TOLLIP, MYD88, and IRAK1 or IRAK2 via the respective TIR domains of the receptor/coreceptor subunits. Binds ligands with comparable affinity and binding of antagonist IL1RN prevents association with IL1RAP to form a signaling complex. Involved in IL1B-mediated costimulation of IFNG production from T-helper 1 (Th1) cells (PubMed:10653850).

Involvement in disease

Chronic recurrent multifocal osteomyelitis 3

CRMO3

An autosomal dominant autoinflammatory bone disease characterized by early-childhood onset of bone pain and arthritis caused by sterile osteomyelitis.

None

The disease may be caused by variants affecting the gene represented in this entry.

Post-translational modifications

A soluble form (sIL1R1) is probably produced by proteolytic cleavage at the cell surface (shedding).

Rapidly phosphorylated on Tyr-496 in response to IL-1, which creates a SH2 binding site for the PI 3-kinase regulatory subunit PIK3R1.

Sequence Similarities

Belongs to the interleukin-1 receptor family.

Tissue Specificity

Expressed in T-helper cell subsets. Preferentially expressed in T-helper 1 (Th1) cells.

Cellular localization

• Membrane
• Single-pass type I membrane protein
• Cell membrane
• Secreted

Alternative names

CD121a, IL1R, IL1RA, IL1RT1, IL1R1, Interleukin-1 receptor type 1, IL-1R-1, IL-1RT-1, IL-1RT1, CD121 antigen-like family member A, Interleukin-1 receptor alpha, Interleukin-1 receptor type I, p80, IL-1R-alpha

Database links

swissprot:P14778 entrezGene:3554 omim:147810

Other research areas

• Immuno-oncology