IL18BP: Interleukin 18 Binding Protein

A key regulator of IL-18 activity in immune and inflammatory responses

Gene Information Card

Symbol IL18BP
Full Name interleukin 18 binding protein
Gene Type protein coding
Chromosomal Location 11q13.4
NCBI Gene ID 10068 ncbi.nlm.nih.gov/gene/10068
Ensembl ID ENSG00000137496
UniProt ID O95998
OMIM ID 604113
HGNC ID 5989
Aliases IL18BP, IL-18BP, IL18BPa, IL18BPb, IL18BPc, IL18BPd

Description

IL18BP encodes the interleukin 18 binding protein, a secreted protein that binds to interleukin 18 (IL-18) with high affinity and neutralizes its activity. It functions as a natural inhibitor of IL-18-mediated immune responses, including interferon-gamma production and Th1 cell activation. Alternative splicing generates multiple isoforms (a, b, c, d) with varying affinities and tissue distributions.

Disease Associations

Disease category Pathophysiological mechanism Genomic evidence
Inflammatory bowel disease (Crohn's disease) Reduced IL18BP levels lead to unopposed IL-18 activity, promoting intestinal inflammation ClinVar, PMID: 20628021
Rheumatoid arthritis Imbalance between IL-18 and IL18BP contributes to synovial inflammation NCBI Gene, PMID: 15208739
Systemic lupus erythematosus Altered IL18BP expression correlates with disease activity OMIM, PMID: 16920933
Sepsis IL18BP modulates IL-18-driven cytokine storm in severe infection UniProt, PMID: 11564765

Expression Profile

Tissue Expression
Tissue nTPM level
Lung 12.5 Medium
Spleen 8.3 Medium
Small intestine 6.1 Low
Colon 5.4 Low
Liver 4.2 Low
Kidney 3.8 Low
Heart 2.1 Not detected
Brain 1.0 Not detected
Cell Line Expression
Cell Line nTPM Notes
THP-1 (monocyte) 15.2 High expression after LPS stimulation
HeLa (cervical) 2.3 Low baseline expression
A549 (lung) 4.1 Moderate expression
HepG2 (liver) 1.8 Low expression
Jurkat (T-cell) 0.9 Not detected
Data source:Human Protein Atlas(proteinatlas.org)

Mutations & Variants

Hotspot Mutations
Variant Type Frequency Functional Description
c.1A>G (p.Met1?) missense <0.01% Potential loss of start codon; likely loss of function
c.200C>T (p.Pro67Leu) missense <0.01% Unknown significance; rare variant
c.340G>A (p.Gly114Arg) missense <0.01% Unknown significance; rare variant
c.500_501insA frameshift <0.01% Predicted loss of function; truncation
Mutation functional classification

Loss of Function (LOF)

Mutations that disrupt the start codon or cause frameshifts/truncations likely reduce or abolish IL-18 binding and neutralization, leading to increased IL-18 activity.

Gain of Function (GOF)

No gain-of-function mutations have been reported in IL18BP.

Dominant Negative (DN)

No dominant-negative mutations have been described for IL18BP.

Pathways

Interleukin-18 signaling pathway (Reactome: R-HSA-448424)
Cytokine-cytokine receptor interaction (KEGG: hsa04060)
NOD-like receptor signaling pathway (KEGG: hsa04621)
Toll-like receptor signaling pathway (KEGG: hsa04620)
Inflammatory bowel disease (KEGG: hsa05321)

Protein Summary

The IL-18 binding protein (IL-18BP) is a secreted glycoprotein of approximately 40 kDa that binds to mature IL-18 with high affinity (Kd ~0.4 nM), preventing IL-18 from interacting with its receptor. It is constitutively expressed in many tissues and upregulated during inflammation. Four major isoforms (a, b, c, d) arise from alternative splicing; isoform a is the most abundant and has the highest affinity. IL-18BP acts as a natural antagonist of IL-18-driven Th1 and NK cell responses, and its dysregulation is implicated in autoimmune and inflammatory diseases.

Related Products

Product name Cat.No. Species Gene ID
IL18BP Knockout HEK293 Cell Line EDJ-KQ2452 Human 10068 Details Get a Quote
IL18BP Knockout HCT 116 Cell Line EDJ-KQ22982 Human 10068 Details Get a Quote
IL18BP Knockout HeLa Cell Line EDJ-KQ22983 Human 10068 Details Get a Quote
IL18BP Knockout A-549 Cell Line EDJ-KQ21648 Human 10068 Details Get a Quote
Displaying Records 1 To 4 Of 4 Records
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