Abstract: Mendelian susceptibility to mycobacterial disease（MSMD）is an inborn immunodeficiency disease, characterizing by selective susceptibility to weakly virulent mycobacteria, such as BCG Vaccine/environmental mycobacteria. IFN-γ plays an important role in antibacterial immune response, and inborn IFN-γ-related immunodeficiency involves in pathogenesis of the MSMD. Since 1996, 18 causal genes of MSMD have been identified，almost all the known genetic etiologies are inborn errors of IFN-γ immunity. Here we review the genetic and immunological features of patients with inborn errors of IFN-γ-dependent immunity in MSMD.

Key words: Mendelian susceptibility to mycobacterial disease, genetic diseases, immunodeficiency, interferon