Human primary immunodeficiencies of type I interferons

E Jouanguy, SY Zhang, A Chapgier, V Sancho-Shimizu… - Biochimie, 2007 - Elsevier
Type I interferons (IFN-α/β and related molecules) are essential for protective immunity to
experimental infection by numerous viruses in the mouse model. In recent years, human
primary immunodeficiencies affecting either the production of (UNC-93B deficiency) or the
response to (STAT1 and TYK2 deficiencies) these IFNs have been reported. Affected
patients are highly susceptible to certain viruses. Patients with STAT1 or TYK2 deficiency
are susceptible to multiple viruses, including herpes simplex virus-1 (HSV-1), whereas UNC …