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TNFRSF13B gene

Reviewed by Medicinsk Expert•Last updated: 2026-10-22•Gene

TNFRSF13B gene

The TNFRSF13B gene provides instructions for making the TACI protein, which is essential for immune system function, B cell maturation, and antibody production.

Normal Function

The TNFRSF13B gene provides instructions for making a protein called TACI. The TACI protein is found on the surface of immune system cells called B cells. These specialized white blood cells help protect the body against infection from foreign invaders such as bacteria and viruses. When B cells mature, they produce special proteins called antibodies (also known as immunoglobulins). Antibodies attach to specific foreign invaders, marking them for destruction. Through interactions with other proteins, TACI promotes cell signaling, plays a role in B cell survival and maturation, and is involved in the production of antibodies.

Health Conditions Related to Genetic Changes

Common variable immune deficiency

More than 25 mutations in the TNFRSF13B gene have been associated with common variable immune deficiency (CVID). This condition impairs the immune system, resulting in increased risk for recurrent infections; autoimmune disorders, which occur when the immune system malfunctions and attacks the body's tissues and organs; and certain cancers.

Most of the TNFRSF13B gene mutations associated with CVID change single protein building blocks (amino acids) in the TACI protein. The most common mutation seen in people with CVID replaces the amino acid cysteine with the amino acid arginine at position 104 in the TACI protein (written as Cys104Arg or C104R). This mutation impairs the ability of TACI to interact with other proteins, disrupting cell signaling and preventing normal B cell maturation and antibody production. A shortage (deficiency) of certain antibodies makes it difficult for people to fight off infections. Abnormal and deficient immune responses over time likely contribute to the increased cancer risk in people with CVID.

Some people with TNFRSF13B gene mutations do not develop the signs and symptoms of CVID. In these individuals, additional genetic or environmental factors are probably needed for the condition to occur.

Other Names for This Gene

  • CD267
  • IGAD2
  • TACI
  • TR13B_HUMAN
  • transmembrane activator and CAML interactor
  • tumor necrosis factor receptor 13B
  • tumor necrosis factor receptor superfamily member 13B
  • tumor necrosis factor receptor superfamily, member 13B

Frequently Asked Questions

TACI mutation increases the risk of common variable immune deficiency (CVID), but not everyone with the mutation develops disease. Testing first‑degree relatives (parents, siblings, children) is advisable, especially if they have a history of recurrent infections, autoimmune issues, or unexplained low antibody levels. A simple blood test to measure immunoglobulin levels and targeted genetic sequencing can identify carriers, allowing early monitoring and preventive care.
TACI mutations impair B‑cell maturation and antibody production, so vaccine‑induced antibody responses can be suboptimal. Nonetheless, vaccines still provide some protection and stimulate other parts of the immune system. Your immunologist may measure specific antibody titers after vaccination and recommend additional booster doses, higher‑dose formulations (e.g., high‑dose flu vaccine), or passive immunoglobulin replacement to ensure adequate protection.
Typical infections include sinusitis, bronchitis, pneumonia, ear infections, and gastrointestinal infections (often with Giardia or viral pathogens). Autoimmune manifestations can involve the gut (celiac‑like disease), blood (immune thrombocytopenia), or joints (arthritis). Seek medical care promptly if you develop fever lasting more than 48 hours, new or worsening sinus or lung symptoms, unexplained joint swelling, or signs of bleeding/bruising. Early treatment reduces complications.
Immunoglobulin can be given intravenously (IVIG) every 3–4 weeks or subcutaneously (SCIG) weekly or bi‑weekly, depending on your lifestyle and tolerance. Common side effects include mild headache, fatigue, or low‑grade fever after IVIG, and local skin redness or swelling with SCIG. Severe reactions are rare but include allergic responses or kidney issues; these are monitored during the first few infusions. Your specialist will tailor the dose to maintain protective IgG levels and adjust the route if side effects become problematic.
Source: MedlinePlus, National Library of Medicine

MedlinePlus brings together authoritative health information from the National Library of Medicine (NLM), the National Institutes of Health (NIH), and other government agencies and health-related organizations.