Immunodeficiency 31A syndrome, also known as IMD31A, is caused by autosomal dominant (AD) STAT1 deficiency.
STAT1 is critical for cellular responses to type I and type III interferons. Autosomal dominant STAT1 deficiency selectively affects the IFNG signaling pathway and confers a predisposition to mycobacterial infections. Furthermore, increased susceptibility to herpes simplex encephalitis (HSE) in adulthood has been reported (Mork et al. 2015).