Article
Human complete Stat-1 deficiency is associated with defective type I and II IFN responses in vitro but immunity to some low virulence viruses in vivo.
Journal of immunology (Baltimore, Md. : 1950) - 15 Apr 2006
Chapgier Ariane, Wynn Robert F, Jouanguy Emmanuelle, Filipe-Santos Orchidée, Zhang Shenying, Feinberg Jacqueline, Hawkins Kay, Casanova Jean-Laurent, Arkwright Peter D
Abstract excerpt
The autosomal recessive form of human complete Stat-1 deficiency is a rare disorder, thus far reported in two unrelated patients, both of whom developed disseminated bacillus Calmette-Guérin (BCG) and subsequently died of viral illnesses before detailed studies of the condition could be performed. It is associated with impaired cellular responses to both IFN-gamma and IFN-alphabeta via Stat-1-containing...
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