LILRA4
Leukocyte immunoglobulin-like receptor A4, a plasmacytoid dendritic cell-specific receptor involved in type I interferon regulation
Gene Information Card
| Symbol | LILRA4 |
|---|---|
| Full Name | Leukocyte immunoglobulin like receptor A4 |
| Gene Type | protein-coding |
| Chromosomal Location | 19q13.42 |
| NCBI Gene ID | 23547 ncbi.nlm.nih.gov/gene/23547 |
| Ensembl ID | ENSG00000197110 |
| UniProt ID | P59901 |
| OMIM ID | 607517 |
| HGNC ID | 14903 |
| Aliases | CD85g, ILT7, LIR-5 |
Description
LILRA4 (Leukocyte immunoglobulin like receptor A4) is a member of the leukocyte immunoglobulin-like receptor (LIR) family. It is selectively expressed on plasmacytoid dendritic cells (pDCs) and functions as an inhibitory receptor. LILRA4 binds to its ligand, bone marrow stromal cell antigen 2 (BST2), and negatively regulates the production of type I interferons (e.g., IFN-α) and pro-inflammatory cytokines. This receptor plays a critical role in modulating innate immune responses and is implicated in autoimmune diseases and cancer.
Disease Associations
| Disease category | Pathophysiological mechanism | Genomic evidence |
|---|---|---|
| Systemic lupus erythematosus (SLE) | LILRA4 signaling via BST2 suppresses IFN-α production; dysregulation may contribute to SLE pathogenesis | ClinVar, OMIM |
| Psoriasis | Altered LILRA4 expression on pDCs may influence IFN-α-driven inflammation | NCBI Gene, PubMed |
| HIV infection | BST2 (tetherin) interaction with LILRA4 modulates pDC IFN responses during viral infection | UniProt, PubMed |
Expression Profile
Tissue Expression
| Tissue | nTPM | level |
|---|---|---|
| Lymph node | 0.0 | Not detected |
| Spleen | 0.0 | Not detected |
| Bone marrow | 0.0 | Not detected |
| Whole blood | 0.0 | Not detected |
| Plasmacytoid dendritic cells (pDCs) | N/A | High (cell-type specific) |
Cell Line Expression
| Cell Line | nTPM | Notes |
|---|---|---|
| pDC (primary) | N/A | High expression; specific to this cell type |
| Monocyte-derived DCs | N/A | Low or absent |
| HEK293 | N/A | Not expressed endogenously |
Data source:Human Protein Atlas(proteinatlas.org)
Mutations & Variants
Hotspot Mutations
| Variant | Type | Frequency | Functional Description |
|---|---|---|---|
| rs11465384 | SNP (intronic) | 0.05 (global) | Associated with altered LILRA4 expression in pDCs; potential link to autoimmune risk |
| rs11150606 | SNP (intronic) | 0.10 (global) | Reported in GWAS for SLE susceptibility |
Mutation functional classification
Loss of Function (LOF)
No confirmed loss-of-function mutations reported in major databases.
Gain of Function (GOF)
No confirmed gain-of-function mutations reported.
Dominant Negative (DN)
Not described.
View complete mutation data:
Gene Ontology (GO)
| • adaptive immune response (GO:0002250) | • immune system process (GO:0002376) |
| • transmembrane signaling receptor activity (GO:0004888) | • signal transduction (GO:0007165) |
| • integral component of membrane (GO:0016021) | • type I interferon production (GO:0032606) |
| • innate immune response (GO:0045087) | • regulation of immune response (GO:0050776) |
Pathways
• Immune System (Reactome: R-HSA-168256)
• Interferon Signaling (Reactome: R-HSA-913531)
• Innate Immune System (Reactome: R-HSA-168249)
Protein Summary
LILRA4 (CD85g/ILT7) is a 499-amino acid transmembrane glycoprotein with four extracellular immunoglobulin-like domains and a long cytoplasmic tail containing immunoreceptor tyrosine-based inhibitory motifs (ITIMs). Upon binding BST2, LILRA4 recruits phosphatases such as SHP-1 and SHP-2, leading to inhibition of FcεRI-mediated signaling and suppression of IFN-α production in pDCs. It is a key negative regulator of pDC function and a potential therapeutic target for autoimmune diseases and cancer immunotherapy.
Related Services
Related Products
| Product name | Cat.No. | Species | Gene ID | |
|---|---|---|---|---|
| LILRA4 Knockout HEK293 Cell Line | EDJ-KQ8057 | Human | 23547 | Details Get a Quote |
| LILRA4 Knockout HeLa Cell Line | EDJ-KQ55766 | Human | 23547 | Details Get a Quote |
| LILRA4 Knockout A-549 Cell Line | EDJ-KQ64262 | Human | 23547 | Details Get a Quote |
| LILRA4 Knockout HCT 116 Cell Line | EDJ-KQ72708 | Human | 23547 | Details Get a Quote |