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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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LILRB4
leukocyte immunoglobulin like receptor B4
Chromosome 19 · 19q13.42
NCBI Gene: 11006Ensembl: ENSG00000186818.13HGNC: HGNC:6608UniProt: A0A0A0MS20
82PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Receptor
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
negative regulation of monocyte activationprotein bindingprotein phosphatase bindingprotein tyrosine kinase inhibitor activityneurodegenerative diseaseneoplasmacute myeloid leukemiaMiyoshi myopathy
✦AI Summary

LILRB4 is an inhibitory immunoglobulin-like receptor that functions as a key negative regulator of immune responses across multiple cell types and disease contexts. As an immune checkpoint molecule expressed on myeloid cells and leukemic blasts, LILRB4 suppresses T cell proliferation and cytotoxic differentiation while promoting immune tolerance 1. The receptor mediates inhibitory signaling through multiple ligands including apolipoprotein E, fibronectin, secretogranin 2, and ALCAM, triggering SHP-2-dependent phosphatase cascades that reduce phosphorylation of signaling proteins and dampen calcium mobilization 12. In monocytes, LILRB4 engagement suppresses Fc receptor-mediated activation and TNF production 1. LILRB4 has emerged as a critical therapeutic target in hematologic and solid malignancies. In acute myeloid leukemia, LILRB4 orchestrates tumor cell invasion and creates an immunosuppressive microenvironment 1. High LILRB4 expression on multiple myeloma cells correlates with poor prognosis and drug resistance, while promoting myeloid-derived suppressive cell function 3. In Alzheimer's disease, microglial LILRB4 suppresses amyloid-β clearance through ApoE binding; anti-LILRB4 antibodies enhance microglial activity and reduce amyloid pathology 4. LILRB4-targeted therapies, including monoclonal antibodies and CAR-T cell approaches, show promise for restoring anti-tumor and anti-pathogen immunity.

Sources cited
1
LILRB4 mediates T cell suppression and tumor infiltration in AML through APOE-dependent signaling; LILRB4 blockade impedes AML development
PMID: 30333625
2
Secretogranin 2 binds LILRB4 on monocytic cells triggering SHP-dependent and SHP-independent STAT3 activation, promoting immunosuppression
PMID: 40707822
3
LILRB4 is highly expressed on AD patient microglia; anti-LILRB4 antibodies reduce amyloid burden and enhance microglial activity by blocking ApoE interaction
PMID: 38569016
4
LILRB4 expression on multiple myeloma cells correlates with poor prognosis; LILRB4 deletion reduces MDSC function and rescues T cell dysfunction
PMID: 38813706
5
LILRB4 is expressed on cytotoxic Tr1 cells that suppress anti-tumor immunity; anti-LILRB4 blockade overcomes this immunosuppression
PMID: 39048822
6
FTO inhibition suppresses LILRB4 expression in leukemia cells, sensitizing them to T cell cytotoxicity
PMID: 32531268
7
LILRB4 promotes prostate cancer progression by modulating NF-κB and PI3K/AKT pathways
PMID: 40576161
Disease Associationsⓘ20
neurodegenerative diseaseOpen Targets
0.25Weak
neoplasmOpen Targets
0.11Weak
acute myeloid leukemiaOpen Targets
0.11Weak
Miyoshi myopathyOpen Targets
0.10Weak
toxoplasmosisOpen Targets
0.09Suggestive
preeclampsiaOpen Targets
0.09Suggestive
multiple myelomaOpen Targets
0.08Suggestive
Alzheimer diseaseOpen Targets
0.08Suggestive
chronic obstructive pulmonary diseaseOpen Targets
0.08Suggestive
lung adenocarcinomaOpen Targets
0.08Suggestive
gastric cancerOpen Targets
0.07Suggestive
hypertrophic cardiomyopathyOpen Targets
0.07Suggestive
posterior cortical atrophyOpen Targets
0.07Suggestive
Rare familial disorder with hypertrophic cardiomyopathyOpen Targets
0.07Suggestive
papillary thyroid carcinomaOpen Targets
0.07Suggestive
Romano-Ward syndromeOpen Targets
0.07Suggestive
non-small cell lung carcinomaOpen Targets
0.07Suggestive
atrial fibrillationOpen Targets
0.07Suggestive
acute lung injuryOpen Targets
0.07Suggestive
Arrhythmogenic right ventricular dysplasiaOpen Targets
0.07Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
CD86Protein interaction100%HLA-AProtein interaction100%PTPN6Protein interaction92%ALCAMProtein interaction81%CD300CProtein interaction81%HLA-EProtein interaction80%
Tissue Expression6 tissues
Bone Marrow
100%
Lung
56%
Brain
17%
Heart
14%
Liver
14%
Ovary
3%
Gene Interaction Network
Click a node to explore
LILRB4CD86HLA-APTPN6ALCAMCD300CHLA-E
PROTEIN STRUCTURE
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PDB3P2T · 1.70 Å · X-ray
View on RCSB ↗
RankingsWhere LILRB4 stands among ~20K protein-coding genes
  • #5,805of 20,598
    Most Researched82
Genes detectedLILRB4
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Neuroinflammation in Alzheimer disease.
PMID: 39653749
Nat Rev Immunol · 2025
1.00
2
Targeting FTO Suppresses Cancer Stem Cell Maintenance and Immune Evasion.
PMID: 32531268
Cancer Cell · 2020
0.90
3
LILRB4 signalling in leukaemia cells mediates T cell suppression and tumour infiltration.
PMID: 30333625
Nature · 2018
0.80
4
Antibody-mediated targeting of human microglial leukocyte Ig-like receptor B4 attenuates amyloid pathology in a mouse model.
PMID: 38569016
Sci Transl Med · 2024
0.70
5
Secretogranin 2 binds LILRB4 resulting in immunosuppression.
PMID: 40707822
Nat Immunol · 2025
0.60