Article
Protein stabilization improves STAT3 function in autosomal dominant hyper-IgE syndrome.
Blood - 29 Dec 2016
Bocchini Claire E, Nahmod Karen, Katsonis Panagiotis, Kim Sang, Kasembeli Moses M, Freeman Alexandra, Lichtarge Olivier, Makedonas George, Tweardy David J
Abstract excerpt
Autosomal dominant hyper-IgE syndrome (AD-HIES) is caused by dominant-negative mutations in STAT3; however, the molecular basis for mutant STAT3 allele dysfunction is unclear and treatment remains supportive. We hypothesized that AD-HIES mutations decrease STAT3 protein stability and that mutant STAT3 activity can be improved by agents that increase chaperone protein activity. We used computer modeling to...
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