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

Details

Serval ID
serval:BIB_CD0F1B410CEC
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Mycobacterial disease and impaired IFN-γ immunity in humans with inherited ISG15 deficiency.
Journal
Science
Author(s)
Bogunovic D., Byun M., Durfee L.A., Abhyankar A., Sanal O., Mansouri D., Salem S., Radovanovic I., Grant A.V., Adimi P., Mansouri N., Okada S., Bryant V.L., Kong X.F., Kreins A., Velez M.M., Boisson B., Khalilzadeh S., Ozcelik U., Darazam I.A., Schoggins J.W., Rice C.M., Al-Muhsen S., Behr M., Vogt G., Puel A., Bustamante J., Gros P., Huibregtse J.M., Abel L., Boisson-Dupuis S., Casanova J.L.
ISSN
1095-9203 (Electronic)
ISSN-L
0036-8075
Publication state
Published
Issued date
28/09/2012
Peer-reviewed
Oui
Volume
337
Number
6102
Pages
1684-1688
Language
english
Notes
Publication types: Case Reports ; Journal Article ; Research Support, N.I.H., Extramural ; Research Support, Non-U.S. Gov't
Publication Status: ppublish
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 the enhanced susceptibility to mycobacterial disease. This experiment of nature shows that human ISGylation is largely redundant for antiviral immunity, but that ISG15 plays an essential role as an IFN-γ-inducing secreted molecule for optimal antimycobacterial immunity.
Keywords
Animals, Antibodies, Viral/blood, Cytokines/genetics, Cytokines/immunology, Female, Granulocytes/immunology, Humans, Immunity, Interferon-gamma/immunology, Interleukin-12/immunology, Killer Cells, Natural/immunology, Male, Mice, Mycobacterium Infections/blood, Mycobacterium Infections/genetics, Mycobacterium Infections/immunology, Pedigree, T-Lymphocytes/immunology, Ubiquitins/genetics, Ubiquitins/immunology, Virus Diseases/blood, Virus Diseases/immunology
Pubmed
Web of science
Create date
04/02/2021 18:57
Last modification date
07/07/2021 5:37
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