Interleukin 1 receptor (type1)

Last updated on: 19.01.2024

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DefinitionThis section has been translated automatically.

The interleukin-1 receptor (type 1) is a cytokine receptor that belongs to the interleukin-1 receptor family. The coding gene IL1R1 is located on the gene loci 2q11.2-q12.1.

General informationThis section has been translated automatically.

The interleukin-1 receptor is an important mediator involved in many cytokine-induced immune and inflammatory responses. Upon binding to its ligand, the cytokine interleukin-1 , the protein complex associates with the coreceptor IL1RAP to form the high-affinity interleukin-1 receptor complex, which mediates interleukin-1-dependent activation of NF-kappaB, MAPK and other signaling pathways. The 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.

The receptor protein also binds ligands with comparable affinity. For example, binding of the interleukin-1 receptor antagonist(IL-1RA) prevents binding with the coreceptor IL-1RAP to form the active signaling complex. Involved in interleukin-1beta-mediated costimulation of IFNgamma production by Th1 cells (Tominaga K et al. 2000)

Diseases associated with mutations of the interleukin-1 receptor protein include:

  • Chronic recurrent multifocal osteomyelitis 3 (CRI3)

and

  • Schnitzler syndrome, a rare, late-onset, non-hereditary (familial) autoinflammatory disease (AID). According to the Strasbourg diagnostic criteria established in 2012 (see below), Schnitzler's syndrome shows a chronic urticarial rash and a monoclonal gammopathy of the IgM and, less frequently, IgG type (85%/15% of cases; mandatory main criteria/de Koning HD 2014). The minor Strasbourg criteria of the disease include recurrent fever, abnormal bone remodeling with or without bone pain, a neutrophilic infiltrate in the skin biopsy and leukocytosis or elevated CRP.

LiteratureThis section has been translated automatically.

  1. Chen S. (2022) et al.Rare mutations in NLRP3 and NLRP12 associated with familial cold autoinflammatory syndrome: two Chinese pedigrees.Clin Rheumatol 41: 3461-3470
  2. Cozzi A et al. (2016) Cutaneous manifestations of adult-onset Still's disease: a case report and review of literature. Clin Rheumatol 35: 1377-1382.
  3. de Koning HD (2014) Schnitzler's syndrome: lessons from 281 cases. Clin Transl Allergy 4: 41
  4. Elling E et al. (2008) Schnitzler's syndrome: chronic urticaria and monoclonal gammopathy-an autoinflammatory syndrome. JDDG 6: 626-631
  5. Mosley B et al. (1987) The interleukin-1 receptor binds the human interleukin-1 alpha precursor but not the interleukin-1 beta precursor. J Biol Chem 262:2941-294
  6. Tominaga K et al. (2000) IL-12 synergizes with IL-18 or IL-1beta for IFN-gamma production from human T cells. Int Immunol 12:151-160.

Last updated on: 19.01.2024