BAFF receptor

BAFF receptor (also known as B-cell activating factor receptor or BAFF-R) is a protein that in humans is encoded by the TNFRSF13C gene. It is a member of the tumor necrosis factor receptor superfamily, which plays a crucial role in B-cell development and autoimmunity.

Function

The BAFF receptor is primarily expressed on mature B lymphocytes, and to a lesser extent on T lymphocytes and dendritic cells. It binds to the B-cell activating factor (BAFF), promoting B-cell survival and proliferation. BAFF and its receptor are essential for the development of mature B cells in the spleen, lymph nodes, and Peyer's patches.

Clinical significance

Mutations in the TNFRSF13C gene can lead to a condition known as common variable immunodeficiency (CVID), characterized by low levels of serum immunoglobulins and increased susceptibility to infections. Additionally, overexpression of BAFF or its receptor has been associated with various autoimmune diseases, including systemic lupus erythematosus (SLE), rheumatoid arthritis, and Sjögren's syndrome.

Therapeutic implications

Given its role in autoimmune diseases, the BAFF receptor has been targeted for therapeutic interventions. Belimumab is a monoclonal antibody that inhibits BAFF and has been approved for the treatment of SLE. Other drugs targeting the BAFF-BAFF-R pathway are currently under investigation.

See also

• Tumor necrosis factor receptor
• B-cell activating factor
• Common variable immunodeficiency
• Systemic lupus erythematosus
• Rheumatoid arthritis
• Sjögren's syndrome