We found a match
Your institution may have access to this item. Find your institution then sign in to continue.
- Title
Jak3, severe combined immunodeficiency, and a new class of immunosuppressive drugs.
- Authors
Pesu, Marko; Candotti, Fabio; Husa, Matthew; Hofmann, Sigrun R; Notarangelo, Luigi D; O'Shea, John J
- Abstract
The recent elucidation of the multiple molecular mechanisms underlying severe combined immunodeficiency (SCID) is an impressive example of the power of molecular medicine. Analysis of patients and the concomitant generation of animal models mimicking these disorders have quickly provided great insights into the pathophysiology of these potentially devastating illnesses. In this review, we summarize the discoveries that led to the understanding of the role of cytokine receptors and a specific tyrosine kinase, Janus kinase 3 (Jak3), in the pathogenesis of SCID. We discuss how the identification of mutations of Jak3 in autosomal recessive SCID has facilitated the diagnosis of these disorders, offered new insights into the biology of this kinase, permitted new avenues for therapy, and provided the rationale for a generation of a new class of immunosuppressants.
- Publication
Immunological reviews, 2005, Vol 203, p127
- ISSN
0105-2896
- Publication type
Journal Article
- DOI
10.1111/j.0105-2896.2005.00220.x