Mycobacterial disease and impaired IFN-γ immunity in humans with inherited ISG15 deficiency.

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 Velez; 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-Dupuis; Jean-Laurent Casanova

Journal article

Mycobacterial disease and impaired IFN-γ immunity in humans with inherited ISG15 deficiency.

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 Velez, Bertrand Boisson, Soheila Khalilzadeh, Ugur Ozcelik, Ilad Alavi Darazam Show all

Science | Published : 2012

DOI: 10.1126/science.1224026

Abstract

ISG15 is an interferon (IFN)-α/β-inducible, ubiquitin-like intracellular protein. Its conjugation to various proteins (ISGylation) contributes to antiviral immunity in mice. Here, we describe human patients with inherited ISG15 deficiency and mycobacterial, but not viral, diseases. The lack of intracellular ISG15 production and protein ISGylation was not associated with cellular susceptibility to any viruses that we tested, consistent with the lack of viral diseases in these patients. By contrast, the lack of mycobacterium-induced ISG15 secretion by leukocytes-granulocyte, in particular-reduced the production of IFN-γ by lymphocytes, including natural killer cells, probably accounting for th..

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