LAIR1
Description
The LAIR1 (leukocyte associated immunoglobulin like receptor 1) is a protein-coding gene located on chromosome 19.
LAIR1, also known as CD305, is an inhibitory receptor found on various immune cells like NK cells, T cells, and B cells. It helps regulate the immune response by preventing the destruction of self-cells. LAIR1 belongs to the immunoglobulin superfamily and the leukocyte-associated inhibitory receptor family. It's located in a region called the leukocyte receptor cluster on chromosome 19, which contains many genes encoding immune receptors. LAIR1 interacts with PTPN11 and PTPN6, two proteins involved in cell signaling.
LAIR1 acts as an inhibitory receptor, suppressing the cytotoxic activity of NK cells, B cells, and T cells. Activation through tyrosine phosphorylation leads to the recruitment and activation of phosphatases PTPN6 and PTPN11. This receptor also reduces intracellular calcium levels triggered by B-cell receptor engagement. Additionally, it might exert its inhibitory function independent of SH2-containing phosphatases. LAIR1 modulates cytokine production in CD4+ T cells, downregulating IL2 and IFNG while inducing TGF-beta secretion. It also downregulates IgG and IgE production in B cells, along with IL8, IL10, and TNF secretion. LAIR1 inhibits proliferation and induces apoptosis in myeloid leukemia cell lines, preventing nuclear translocation of NF-kappa-B p65 subunit/RELA and phosphorylation of I-kappa-B alpha/CHUK in these cells. It further inhibits the differentiation of peripheral blood precursors into dendritic cells.
LAIR1 is also known as CD305, LAIR-1.
Associated Diseases
- cancer
- common variable immunodeficiency
- breast cancer
- isolated agammaglobulinemia
- severe combined immunodeficiency due to IKK2 deficiency
- Wiskott-Aldrich syndrome
- neutrophil immunodeficiency syndrome
- immunodeficiency 72 with autoinflammation
- combined immunodeficiency with skin granulomas
- immunodeficiency 18
- severe combined immunodeficiency due to CTPS1 deficiency