Igα deficiency

Igα deficiency is an autosomal recessive disorder caused by mutation in the Igα (CD79α) leading to B cell defect. Mutations in the gene for Igα as well as in other pre-B cell receptor complex as λ 5/14.1 (IGLL1) and in the the gene for μ heavy chain (IGHM), can cause a disorder clinically similar to XLA.

Alternative names

CD79A deficiency, IGA deficiency

Classification

  • Deficiencies predominantly affecting antibody production
    • Agammaglobulinemia

Inheritance

Autosomal recessive