LILRA4
Description
The LILRA4 (leukocyte immunoglobulin like receptor A4) is a protein-coding gene located on chromosome 19.
LILRA4, also known as CD85g or ILT-7, is a cell surface protein encoded by the LILRA4 gene. It is predominantly expressed in plasmacytoid dendritic cells (PDCs) and is downregulated by interleukin 3 (IL3). LILRA4 is one of 19 related genes forming a leukocyte immunoglobulin-like receptor gene cluster (LRC) on chromosome 19q13.4.
LILRA4 functions as a coreceptor to limit innate immune responses to viral infections. Signaling occurs through interaction with FCER1G, leading to phosphorylation of Src family and Syk kinases and subsequent intracellular Ca(2+) mobilization. LILRA4 downregulates the production of interferons (IFNA1, IFNA2, IFNA4, IFNB1, and TNF) in plasmacytoid dendritic cells exposed to influenza virus or CpG dinucleotides, acting as a negative regulator of TLR7 and TLR9 signaling cascades. It also downregulates interferon production in response to interaction with BST2 on HIV-1 infected cells. LILRA4 does not interfere with the differentiation of plasmacytoid dendritic cells into antigen-presenting cells.
LILRA4 is also known as CD85g, ILT7.
Associated Diseases
- systemic lupus erythematosus
- acquired idiopathic inflammatory myopathy
- discoid lupus erythematosus
- common variable immunodeficiency
- isolated agammaglobulinemia
- classic Hodgkin lymphoma
- severe combined immunodeficiency due to CARD11 deficiency
- severe combined immunodeficiency due to CTPS1 deficiency
- combined immunodeficiency with skin granulomas
- reticular dysgenesis
- immunodeficiency 18
- BENTA disease
- hyper-IgM syndrome type 3
- T-B+ severe combined immunodeficiency due to JAK3 deficiency
- immunodeficiency, common variable, 14
