Serves as a receptor for various ligands including complement component CD3d, HNRNPU OR IFNA1 (PubMed:1849076, PubMed:21527715, PubMed:7753047). When C3d is bound to antigens, attaches to C3d on B-cell surface and thereby facilitates the recognition and uptake of antigens by B-cells (PubMed:21527715). This interaction enhances B-cell activation and subsequent immune responses. Forms a complex with several partners on the surface of B-cells including CD19, FCRL5 and CD81, to form the B-cell coreceptor complex that plays a crucial role in B-cell activation and signaling (PubMed:1383329, PubMed:30107486). Induces also specific intracellular signaling separately from the BCR and CD19 by activating the tyrosine kinase SRC, which then phosphorylates nucleolin/NCL and triggers AKT and GSK3 kinase activities in a SYK/CD19-independent manner (PubMed:12938232). Acts as a ligand for CD23 (FcepsilonRII), a low-affinity receptor for IgE, which is expressed on B-cells and other immune cells, and thus participates in the regulation of IgE production (PubMed:1386409).
(Microbial infection) Acts as a receptor for Epstein-Barr virus.
Systemic lupus erythematosus 9
SLEB9
A chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue, characterized principally by involvement of the skin, joints, kidneys and serosal membranes. It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system. The disease is marked by a wide range of system dysfunctions, an elevated erythrocyte sedimentation rate, and the formation of LE cells in the blood or bone marrow.
None
Disease susceptibility is associated with variants affecting the gene represented in this entry.
Immunodeficiency, common variable, 7
CVID7
A primary immunodeficiency characterized by antibody deficiency, hypogammaglobulinemia, recurrent bacterial infections and an inability to mount an antibody response to antigen. The defect results from a failure of B-cell differentiation and impaired secretion of immunoglobulins; the numbers of circulating B-cells is usually in the normal range, but can be low.
None
The disease is caused by variants affecting the gene represented in this entry.
Belongs to the receptors of complement activation (RCA) family.
Mature B-lymphocytes, T-lymphocytes, pharyngeal epithelial cells, astrocytes and follicular dendritic cells of the spleen.
Proteins
Immunology & Infectious Disease
119160Da
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