Human primary immunodeficiencies of type I interferons

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 defi...

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Bibliographic Details
Published in:Biochimie 2007-06, Vol.89 (6), p.878-883
Main Authors: Jouanguy, Emmanuelle, Zhang, Shen-Ying, Chapgier, Ariane, Sancho-Shimizu, Vanessa, Puel, Anne, Picard, Capucine, Boisson-Dupuis, Stéphanie, Abel, Laurent, Casanova, Jean-Laurent
Format: Article
Language:English
Subjects:
Animals
Antiviral Agents - chemistry
Genetic Predisposition to Disease
Herpes simplex virus 1
Herpes virus
Herpesviridae - metabolism
Herpesvirus 1, Human - metabolism
Human genetics
Humans
Immune deficiency
Immune System
Immunity, Innate
Immunologic Deficiency Syndromes - immunology
Immunologic Deficiency Syndromes - virology
Infectious diseases
Interferons
Membrane Transport Proteins - metabolism
Models, Biological
Prognosis
STAT1 Transcription Factor - metabolism
TYK2 Kinase - metabolism
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Summary: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-93B-deficient patients present isolated HSV-1 encephalitis. However, these immunological defects are not limited to type I IFN-mediated immunity. Impaired type II IFN (IFN-γ)-mediated immunity plays no more than a minor role in the pathogenesis of viral diseases in these patients, but the contribution of impaired type III IFN (IFN-λ)-mediated immunity remains to be determined. These novel inherited disorders strongly suggest that type I IFN-mediated immunity is essential for protection against natural infections caused by several viruses in humans.
ISSN:0300-9084
1638-6183
DOI:10.1016/j.biochi.2007.04.016