CD83
Description
The CD83 (CD83 molecule) is a protein-coding gene located on chromosome 6.
CD83, encoded by the CD83 gene, is a human protein with a complex structure. It exists in both membrane-bound and soluble forms. The membrane-bound form comprises an extracellular V-type immunoglobulin-like domain, a transmembrane domain, and a cytoplasmic signaling tail, forming trimers. The soluble form consists only of the immunoglobulin-like domain and can assemble into dodecameric complexes. The CD83 gene resides on human chromosome 6p23 and is regulated by various factors including NF-κB, interferon regulatory factors, and the aryl hydrocarbon receptor. The membrane-bound form of CD83 stabilizes MHC II, costimulatory molecules, and CD28 in the membrane by inhibiting MARCH-family E3 ubiquitin ligases. While the specific ligands for CD83 are not yet fully understood, the membrane-bound form may interact with the soluble form, suggesting an autocrine immune regulatory mechanism.
CD83 is also known as BL11, HB15.
Associated Diseases
- graft versus host disease
- common variable immunodeficiency
- isolated agammaglobulinemia
- chronic graft versus host disease
- severe combined immunodeficiency due to CARD11 deficiency
- combined immunodeficiency with skin granulomas
