BAFF-R

B-cell activating factor receptor (BAFF-R), also known as BLyS receptor 3 (BR3), is a protein that in humans is encoded by the TNFRSF13C gene. BAFF-R is a member of the tumor necrosis factor receptor superfamily and is a crucial regulator in the development and function of B cells, a type of white blood cells involved in the immune system's response to infection and disease.

Function

BAFF-R is primarily expressed on the surface of B cells. It binds to its ligand, the B-cell activating factor (BAFF), also known as BLyS, which is a crucial survival factor for B cells. The interaction between BAFF and BAFF-R promotes B cell proliferation and survival, playing a vital role in B cell maturation and immunoglobulin production. This receptor-ligand interaction is essential for maintaining the normal operation of the adaptive immune system.

Clinical Significance

Alterations in the BAFF/BAFF-R pathway have been implicated in the pathogenesis of various autoimmune diseases, including systemic lupus erythematosus (SLE), rheumatoid arthritis (RA), and Sjögren's syndrome. Elevated levels of BAFF have been observed in patients with these conditions, suggesting that abnormal BAFF signaling through BAFF-R may contribute to the excessive survival of autoreactive B cells, leading to autoimmunity.

In addition, therapeutic strategies targeting the BAFF/BAFF-R interaction are being explored as potential treatments for autoimmune diseases. For example, Belimumab, a monoclonal antibody that inhibits BAFF, has been approved for the treatment of SLE.

Genetics

The TNFRSF13C gene, encoding the BAFF-R protein, is located on chromosome 22 in humans. Genetic variations in TNFRSF13C have been studied for their potential association with susceptibility to autoimmune diseases, although results have been mixed.

See Also

• B cell
• Tumor necrosis factor superfamily
• Autoimmune disease
• Belimumab