IL-1 signaling pathway

The interleukin-1 (IL-1) signaling pathway is the principal mediator of sterile, innate-immune-driven inflammation in aged tissue. It transmits signals from two potent pro-inflammatory cytokines — IL-1β and IL-1α — through a shared receptor complex (IL-1R1 / IL-1RAcP) into the MyD88 → IRAK4 → TRAF6 → TAK1 → NF-κB / MAPK cascade. The pathway is tonically opposed by the endogenous antagonist IL-1Ra and the decoy receptor IL-1R2. Three FDA-approved biologics target this pathway, and the CANTOS trial (Ridker et al. 2017, n=10,061) established that pharmacological IL-1β blockade reduces cardiovascular events in humans, making this the most-validated therapeutic target within inflammaging 1.

This page is the integrating pathway page for the full IL-1 axis. Cytokine-specific biology lives on the atomic protein pages: il-1b, il-1a, il-1ra, il-1r1, il-18. Per-compound PK and trial data lives on canakinumab and the implicit stubs anakinra, rilonacept.


IL-1 family taxonomy

The IL-1 family currently has 11 members sharing structural homology (beta-trefoil fold) and, for most members, a shared receptor scaffold architecture 2 3:

CytokineGeneReceptor complexSignal outcomeNotes
IL-1αIL1AIL-1R1 + IL-1RAcPNF-κB / MAPKConstitutive; alarmin; SASP apex regulator; also nuclear (intracrine)
IL-1βIL1BIL-1R1 + IL-1RAcPNF-κB / MAPKRequires inflammasome processing; primary pathological ligand in inflammaging
IL-1RaIL1RNIL-1R1 (no IL-1RAcP)None (pure antagonist)Endogenous brake; basis of anakinra
IL-18IL18IL-18R1 + IL-18RAPNF-κB / AP-1 → IFN-γCaspase-1 activated; NK/T-cell IFN-γ inducer
IL-33IL33ST2 (IL1RL1) + IL-1RAcPNF-κB / MAPKAlarmin; chromatin-associated; TH2 bias; senescence relevance emerging
IL-36αIL36AIL-36R + IL-1RAcPNF-κBSkin-dominant; weaker systemic aging role
IL-36βIL36BIL-36R + IL-1RAcPNF-κBSkin-dominant
IL-36γIL36GIL-36R + IL-1RAcPNF-κBSkin-dominant
IL-36RaIL36RNIL-36R (no IL-1RAcP)None (antagonist)Decoy for IL-36 axis
IL-37IL37IL-18Rα (complex)Anti-inflammatoryUniquely suppressive; attenuates innate responses
IL-38IL38IL-36R / IL-1RAPL2Anti-inflammatoryRelatively uncharacterised

The aging-relevant members (bold) are IL-1α, IL-1β, IL-1Ra, and IL-18 — all of which have verified atomic pages in this wiki. IL-33 has emerging senescence relevance (released from senescent fibroblast nuclei upon lytic death) but is not yet seeded. needs-canonical-id — IL-33, IL-36 family, and IL-37 pages are implicit stubs.


Receptor architecture

IL-1R1 — the signalling receptor

IL-1R1 (UniProt P14778; gene IL1R1) is the primary signal-transducing receptor. It is a single-pass type I membrane glycoprotein with three extracellular Ig-like C2-type domains (D1–D3) for ligand binding and an intracellular TIR (Toll/IL-1 Receptor homology) domain for adaptor recruitment.

Ligand ranking for IL-1R1 binding affinity: IL-1β ≈ IL-1α >> IL-1Ra. Despite similar receptor affinity, IL-1Ra fails to recruit the co-receptor IL-1RAcP — this is the structural basis of its antagonism 4.

IL-1RAcP — the co-receptor

IL-1RAcP (IL-1 receptor accessory protein; gene IL1RAP; UniProt Q9NPH3) is the obligate co-receptor for IL-1R1 signalling. It is recruited to the IL-1R1·ligand complex and contributes its own TIR domain to form the two-TIR composite surface that recruits MyD88. IL-1RAcP is also the co-receptor for IL-33 (with ST2) and IL-36 signalling — making it a shared scaffold across three IL-1 family sub-axes.

IL-1R2 — the decoy receptor

IL-1R2 (gene IL1R2; UniProt P27930) is a decoy receptor that binds IL-1β with high affinity but has a truncated cytoplasmic tail incapable of signalling. It functions primarily to sequester IL-1β (and to a lesser degree IL-1α) in the extracellular space, reducing the available pool for IL-1R1 engagement. IL-1R2 is shed from the cell surface as a soluble decoy receptor (sIL-1R2), whose circulating levels increase as part of the regulatory anti-inflammatory response. needs-replication — quantitative data on how sIL-1R2 changes with age in human plasma are limited to small cross-sectional studies.

Soluble receptors

Both IL-1R1 and IL-1R2 undergo proteolytic ectodomain shedding. Soluble forms (sIL-1R1; sIL-1R2) retain ligand-binding capacity and contribute to buffering extracellular IL-1 tone, but their relative quantitative contributions in aged tissue are not established 3. needs-replication


Signal transduction

The canonical IL-1 transduction cascade proceeds from receptor engagement to NF-κB and MAPK activation through a conserved adaptor chain, confirmed by Reactome pathway R-HSA-9020702 5 3:

IL-1α or IL-1β
  → binds IL-1R1 (extracellular domains D1–D2)
  → IL-1RAcP recruited (forms ternary signalling complex)
  → cytoplasmic TIR domains of IL-1R1 + IL-1RAcP brought into proximity
  → MyD88 (adaptor; [[myd88]]) recruited via TIR–TIR interaction (C-terminus of MyD88 binds the IL-1R1/IL-1RAcP heterocomplex)
  → MyD88 mediates IRAK recruitment to the receptor complex (IRAK4 and IRAK1/2; [[irak4]])
  → IRAK4 autophosphorylation → phosphorylates IRAK1 and IRAK2
  → IRAK1/2 dissociate from receptor complex
  → TRAF6 (E3 ubiquitin ligase; [[traf6]]) recruited
  → TRAF6 catalyses K63-linked polyubiquitin chains on itself and IRAK1
  → TAK1 (MAP3K7) + TAB1/2 complex activated by K63-ubiquitin scaffold
      ├─ IKK arm: TAK1 → IKKβ (within IKK complex: IKKα/IKKβ/NEMO)
      │     → IκBα phosphorylation (Ser32/Ser36) → proteasomal degradation
      │     → NF-κB (p65/p50) nuclear translocation → pro-inflammatory gene transcription
      └─ MAPK arm: TAK1 → MKK3/6 → p38 MAPK
                         → MKK4/7 → JNK
                         → AP-1 activation; cytokine mRNA stabilisation

Adaptor pages (R28): myd88, irak4, traf6, and tak1 are now verified atomic protein pages with full mechanistic detail. The signal transduction arrows above are anchored to those atomic pages 5.

PI3K arm

IL-1R1 Tyr496 phosphorylation recruits the p85 regulatory subunit of PI3-kinase → Akt activation, integrating IL-1 signalling with the pi3k-akt-pathway and mtor axes 6. This arm is less well-characterised in the aging context; its contribution to inflammaging is likely minor relative to the NF-κB arm.


Negative regulation

Four major regulatory mechanisms oppose IL-1 signal output:

MechanismEffectorModeNotes
Competitive antagonismIL-1Ra (IL1RN)Occupies IL-1R1 without recruiting IL-1RAcP; no signalRequires ~100–1000× molar excess over IL-1β; basis of anakinra
Decoy receptor sequestrationIL-1R2High-affinity IL-1β/α binding; no intracellular TIR; no signalSoluble shed form (sIL-1R2) extends decoy capacity into extracellular space
Signalling inhibitorSIGIRR (TIR8; IL1RL2)Single Ig-domain protein; recruits IRAK and TRAF6 away from productive signallingExpressed highly in gut epithelium; loss accelerates colitis
Ubiquitin editingA20 (TNFAIP3)Removes K63-ubiquitin from TRAF6; dampens downstream kinase activationShared with TNF/TLR/NF-κB pathways; age-associated decline in A20 expression proposed

IL-1Ra is the dominant physiological regulator and the one best-validated therapeutically. See il-1ra for the full Il1rn biology and anakinra data.


Aging connection

IL-1β as the central SASP cytokine

IL-1β is the single most-studied cytokine in the senescence-associated secretory phenotype (SASP). Senescent cells constitutively activate the NLRP3 inflammasome through accumulated cytosolic DNA and mitochondrial ROS, generating continuous caspase-1 activity and IL-1β output 3. This IL-1β acts on IL-1R1 in neighbouring non-senescent cells, activating NF-κB and propagating the inflammatory state — a paracrine inflammaging mechanism.

IL-1β output from senescent cells is also amplified by IL-1α in an autocrine loop: IL-1α (constitutively expressed on senescent cell surfaces) signals through IL-1R1 → NF-κB → more IL-6, IL-8, MMP production, which further upregulates IL-1β transcription. This IL-1α/IL-1β autocrine-paracrine amplification loop is the proposed molecular engine of SASP maintenance (see il-1a for the primary evidence).

Hematopoietic stem cell inflammaging via IL-1

Chronic low-level IL-1 signalling drives myeloid-biased differentiation of HSCs in aged bone marrow. Kovtonyuk et al. (2022, Blood) showed that microbiota-derived signals elevate steady-state IL-1α/β in aged mouse bone marrow; IL-1R1-deficient HSCs (or antibiotic-treated mice) maintained balanced lymphoid/myeloid output 7. This implicates the IL-1 pathway in the clonal myeloid bias that precedes clonal hematopoiesis and CHIP. For the full CHIP/HSC aging context see chronic-inflammation and the implicit stubs hematopoietic-stem-cells, clonal-hematopoiesis.

DimensionStatus
Pathway conserved in humans?yes — IL-1R1, IL1RAP, MYD88, IRAK1/4, TRAF6 all conserved with high homology
Phenotype (inflammaging) conserved in humans?yes — CANTOS demonstrated the IL-1β limb causally mediates cardiovascular events in humans 1
Replicated in humans?yes (CANTOS, n=10,061); partial (HSC myeloid bias — mechanistic data primarily murine)

CANTOS and the aging-context druggability rationale

The CANTOS trial 1 randomised 10,061 post-MI patients with hsCRP ≥2 mg/L to canakinumab (50, 150, or 300 mg q3mo SC) vs placebo. The 150 mg arm reduced MACE (HR 0.85, 95% CI 0.74–0.98; P=0.021). No LDL reduction was observed — establishing that the CV benefit is inflammation-mediated, not lipid-mediated. This is the single most powerful human proof-of-concept for the IL-1 pathway as a geroprotective target.

Druggability-tier rationale (tier 1): Per CLAUDE.md pathway-schema, the aging-context tier reflects whether a clinical drug exists for an aging indication engaging this pathway. Three FDA-approved IL-1 modulators exist (anakinra, canakinumab, rilonacept), and CANTOS constitutes an aging-relevant Phase 3 trial (post-MI patients selected on inflammatory biomarker; outcome is cardiovascular aging, not a rare genetic syndrome). Tier 1 is justified. See canakinumab for per-arm effect sizes; il-1ra for anakinra compound data.


Therapeutic landscape

AgentMechanismSpecificityApproved indicationAging relevance
Anakinra (Kineret)Recombinant IL-1Ra; competitive IL-1R1 antagonistIL-1R1 — blocks IL-1α and IL-1β equallyRA, CAPS, SJIA, NOMIDT2D (Larsen 2007 NEJM, n=70); HFpEF (D-HART, n=12); short half-life (4–6 h) requires daily SC injection
canakinumab (Ilaris)Anti-IL-1β IgG1κ mAbIL-1β-specific; does not block IL-1αCAPS, SJIA, TRAPS, FMF, goutCANTOS (n=10,061): HR 0.85 at 150 mg; cost/safety barrier to population use 1
Rilonacept (Arcalyst)IL-1 Trap (Fc–IL-1R1–IL-1RAcP fusion)Sequesters IL-1α + IL-1β; similar breadth to anakinraCAPS, recurrent pericarditisNo large aging-specific trial; long half-life (~7 days) better than anakinra; no CANTOS-equivalent
Bermekimab (investigational)Anti-IL-1α mAbIL-1α-specificPhase 2/3 (hidradenitis suppurativa, atopic dermatitis)Tests the IL-1α → SASP apex hypothesis; no aging RCT yet
Isunakinra (investigational)Anti-IL-1R1 mAbBlocks IL-1R1 directlyPhase 1–2 (asthma)Would block all IL-1R1 ligands; not yet aging-focused

CANTOS lessons for pathway-level therapeutics:

  1. IL-1β–specific blockade is sufficient to reduce MACE in a high-inflammaging population (hsCRP ≥2 mg/L), but the effect size is modest (HR 0.85) and the benefit is offset by a higher rate of fatal infection (incidence rate ratio ~1.7 vs placebo: 0.31 vs 0.18 events per 100 person-years in pooled canakinumab vs placebo; P=0.02).
  2. Broad IL-1 blockade (anakinra) suppresses both IL-1α and IL-1β; this may enhance efficacy in SASP-driven contexts (where IL-1α is the apex regulator) but carries a wider immunosuppression risk.
  3. No senolytic intersection trial has combined IL-1 pathway blockade with senescent cell clearance (e.g., fisetin or navitoclax). This is a proposed next experimental step. needs-human-replication

Cross-references

  • il-1b — primary pathological ligand; NLRP3-processed; CANTOS target
  • il-1a — alarmin; constitutive; SASP apex regulator; nuclear intracrine activity
  • il-1ra — endogenous antagonist; basis of anakinra
  • il-1r1 — primary signalling receptor; ligand selectivity; TIR domain
  • il-18 — IL-1 family member with distinct receptor and IFN-γ–inducing role
  • canakinumab — anti-IL-1β mAb; CANTOS trial data canonical home
  • nlrp3-inflammasome — upstream source of processed IL-1β; verified pathway page
  • caspase-1 — protease that activates pro-IL-1β and pro-IL-18
  • nf-kb — primary downstream transcriptional effector arm
  • ras-mapk — parallel MAPK arm (p38, JNK) downstream of TAK1
  • sasp — major functional output of chronic IL-1 signalling in senescent cells
  • chronic-inflammation — hallmark MOC; IL-1 pathway is a core molecular driver
  • cellular-senescence — SASP-derived IL-1α/β sustains senescent state via IL-1R1 → NF-κB loop

Adaptor cascade (verified R28):

  • myd88 — universal IL-1R/TLR adaptor; Myddosome 6:4:4 (MyD88:IRAK4:IRAK2)
  • irak4 — master serine/threonine kinase; first kinase in the IL-1 signalling cascade
  • traf6 — E3 ubiquitin ligase; K63-polyubiquitin scaffold
  • tak1 — MAP3K7; proximal to IKK and MAPK arms

Implicit stubs created by this page:


Limitations and gaps

  • WikiPathways ID: Not retrieved (403 error during seeding). Populate wikipathways: on next lint pass using the WikiPathways REST API or browser lookup for “IL-1 signaling human.” Tag: needs-canonical-id
  • KEGG precision: hsa04060 is the cytokine-cytokine receptor interaction map, not an IL-1-specific intracellular cascade entry. Reactome R-HSA-9020702 is more specific for the transduction cascade and should be the primary reference. If a dedicated KEGG IL-1 signalling pathway entry exists (check KEGG PATHWAY + hsa + search “interleukin-1”), update the kegg: field. needs-canonical-id
  • Reactome ID conflict in nf-kb: The nf-kb.md page incorrectly lists reactome: R-HSA-9020702; this ID belongs to IL-1 signaling. Correct the nf-kb.md Reactome field on next lint pass (but do not modify other pages in this seeding pass).
  • MyD88/IRAK4/TRAF6/TAK1 (resolved R28-2026-05-07): All four adaptor protein pages are now seeded and verified. Signal transduction wikilink graph is complete.
  • IL-1α / SASP apex claim: The claim that IL-1α is the “apex regulator” of the SASP relies on Orjalo et al. 2009 (verified on il-1a). That verification is recorded on the atomic page; the pathway page inherits the claim by wikilink without repeating the evidence. No additional gap.
  • IL-1R2 aging data: Quantitative data on age-associated changes in sIL-1R2 levels are sparse; the decoy receptor’s contribution to IL-1 buffering in aged tissue is incompletely characterised. needs-replication
  • Senolytic intersection: No published trial combining IL-1 pathway blockade with senolytic clearance (navitoclax, fisetin). This remains an important next experiment. needs-human-replication
  • Hematopoietic aging claim: Kovtonyuk 2022 (Blood) is a mouse study; the microbiome–IL-1–HSC axis in human aging is inferred but not directly tested. needs-human-replication
  • IL-33 in senescence: Emerging evidence that IL-33 is released from senescent cell nuclei on lytic death (alarmin function); no atomic page yet. needs-replication; il-33 is an implicit stub.

Footnotes

Footnotes

  1. doi:10.1056/NEJMoa1707914 · Ridker PM et al. · NEJM 2017 · rct · n=10,061 (placebo n=3344; 50 mg n=2170; 150 mg n=2284; 300 mg n=2263) · post-MI patients with hsCRP ≥2 mg/L; canakinumab 50/150/300 mg q3mo SC vs placebo; median follow-up 3.7 yr; primary endpoint MACE; 150 mg arm HR 0.85 (95% CI 0.74–0.98; P=0.021); hsCRP −37 pp vs placebo at 48 months (150 mg arm); no LDL change; fatal infection incidence rate ratio ~1.7 (0.31 vs 0.18 events/100 person-years; P=0.02); local PDF: — canonical CANTOS numerics on canakinumab 2 3 4

  2. doi:10.1111/imr.12621 · Dinarello CA · Immunol Rev 2018 · review · IL-1 family overview including all 11 members, receptor architecture, TIR-domain signalling, and negative regulation; ~1731 citations; OA (green PMC5756628); DOI lookup failed — not locally available

  3. doi:10.1182/blood-2010-07-273417 · Dinarello CA · Blood 2011 · review · comprehensive coverage of IL-1β central role in acute and chronic inflammation; TIR-domain signalling; therapeutic neutralisation; ~2149 citations; OA (bronze); not yet locally downloaded 2 3 4

  4. doi:10.1074/jbc.270.23.13757 · Greenfeder SA et al. · J Biol Chem 1995 · in-vitro (molecular cloning, binding studies) · cloned IL-1RAcP as the obligate co-receptor for IL-1R1 signalling; showed IL-1Ra occupies IL-1R1 without recruiting IL-1RAcP; structural basis of antagonism; ~638 citations; local PDF available

  5. doi:10.1016/s1074-7613(00)80402-1 · Wesche H et al. · Immunity 1997 · in-vitro (co-immunoprecipitation, kinase assay) · identified MyD88 as the TIR-domain adaptor recruited to IL-1R1 upon ligand binding; showed MyD88 recruits IRAK to the receptor complex; ~1104 citations; local PDF available 2

  6. Cross-reference to il-1r1 atomic page (verified 2026-05-06) for IL-1R1 Tyr496 phosphorylation → PI3K-p85 interaction claim; canonical source is Boraschi & Tagliabue 2013 (doi:10.1016/j.smim.2013.10.023) as cited there

  7. doi:10.1182/blood.2021011570 · Kovtonyuk LV et al. · Blood 2022 · in-vivo (mouse) · microbiota-derived signals elevate steady-state IL-1α/β in aged bone marrow; IL-1R1 KO HSCs maintain balanced lymphomyeloid output; antibiotic suppression phenocopies; ~135 citations; OA (hybrid); not yet locally downloaded; needs-human-replication