Igβ deficiency

Igβ mutations is involved in a failure of B cell development and can provide valuable insight into the assembly and function of BCR. The majority of patients with the early onset of infection, panhypogammaglobulinemia, reduced or absent B cells (over 85%) are males with mutations in BTK. 5% of patients have defects in the μ H chain, λ5, Igα or BLNK.

Alternative names


Immunoglobulin-associated beta; IGB

Immunoglobulin-associated b29 protein; B29


  • Deficiencies predominantly affecting antibody production
    • Agammaglobulinemia


Autosomal recessive