Mycobacterial Disease and Impaired IFN-γ Immunity in Humans with Inherited ISG15 Deficiency

• DOI: 10.1126/science.1224026
• Publication Date: 2012-08-03T04:37:22Z
• Authors: Dusan Bogunovic; Minji Byun; Larissa A. Durfee; Avinash Abhyankar; Ozden Sanal; Davood Mansouri; Sandra Salem; Irena Radovanovic; Audrey V. Grant; Parisa Adimi; Nahal Mansouri; Satoshi Okada; Vanessa L. Bryant; Xiao-Fei Kong; Alexandra Kreins; Marcela Moncada V...; Bertrand Boisson; Soheila Khalilzadeh; Ugur Ozcelik; Ilad Alavi Darazam; John W. Schoggins; Charles M. Rice; Saleh Al-Muhsen; Marcel Behr; Guillaume Vogt; Anne Puel; Jacinta Bustamante; Philippe Gros; Jon M. Huibregtse; Laurent Abel; Stéphanie Boisson...; Jean-Laurent Casa...
• Keywords: ISG15

Tuberculosis Vaccine Conundrum Some children experience severe clinical disease when they are vaccinated against tuberculosis, an attenuated live vaccine that is normally innocuous in humans. Several germline mutations have been identified account for this susceptibility, and now Bogunovic et al. (p. 1684 , published online 2 August) add another to the list— ISG15 . Uncovering mutation, which inherited autosomal recessive manner, was a surprise because studies with mice deficient showed enhanced susceptibility some viral, but not bacterial, infections. Nevertheless, patients lacking were able produce adequate amounts of interferon-γ, cytokine critical clearance bacteria.