Jak3 and the pathogenesis of severe combined immunodeficiency

Détails

ID Serval
serval:BIB_8D9302364336
Type
Article: article d'un périodique ou d'un magazine.
Sous-type
Synthèse (review): revue aussi complète que possible des connaissances sur un sujet, rédigée à partir de l'analyse exhaustive des travaux publiés.
Collection
Publications
Titre
Jak3 and the pathogenesis of severe combined immunodeficiency
Périodique
Mol Immunol
Auteur(s)
O'Shea J. J., Husa M., Li D., Hofmann S. R., Watford W., Roberts J. L., Buckley R. H., Changelian P., Candotti F.
ISSN
0161-5890 (Print)
ISSN-L
0161-5890
Statut éditorial
Publié
Date de publication
07/2004
Volume
41
Numéro
6-7
Pages
727-37
Langue
anglais
Notes
O'Shea, John J
Husa, Matthew
Li, Denise
Hofmann, Sigrun R
Watford, Wendy
Roberts, Joseph L
Buckley, Rebecca H
Changelian, Paul
Candotti, Fabio
eng
Review
England
Mol Immunol. 2004 Jul;41(6-7):727-37.
Résumé
The discovery that Jak3 mutations are a significant cause of severe combined immunodeficiency (SCID), a rare inherited defect characterized by lymphopenia, has provided valuable insights into the functions of Jak3 in lymphoid development and function. The current therapy for patients suffering from Jak3 SCID is hematopoetic stem cell transplantation, although gene therapy trials have also been performed. In lieu of crystal structure data, these patient-derived mutations have aided in the elucidation of the functions of various structural components of Jak3. By virtue of its requirement for lymphoid functions, Jak3 makes a tantalizing target for immunosuppression and anti-cancer therapy. Herein, we discuss the normal actions of the gammac cytokines, the pathogenesis and treatment protocols for SCID, and finally, the production of a new, selective Jak3 inhibitor capable of preventing transplant rejection in two animal models. Further study of Jak3 will hopefully provide insights into the clinical treatment of patients suffering from immune-mediated diseases.
Mots-clé
Animals, Cytokines/genetics/metabolism, Humans, Janus Kinase 3, Mutation, Protein-Tyrosine Kinases/antagonists & inhibitors/*deficiency/genetics, Receptors, Interleukin-7/genetics/metabolism, Severe Combined Immunodeficiency/enzymology/*genetics/immunology, Signal Transduction/immunology/*physiology, Structure-Activity Relationship
Pubmed
Création de la notice
01/11/2017 11:29
Dernière modification de la notice
03/03/2018 19:16
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