Article
Fatal Lymphoproliferative Disease in Two Siblings Lacking Functional FAAP24.
Journal of clinical immunology - 1 Oct 2016
Daschkey Svenja, Bienemann Kirsten, Schuster Volker, Kreth Hans Wolfgang, Linka René Martin, Hönscheid Andrea, Fritz Gerhard, Johannes Christian, Fleckenstein Bernhard, Kempkes Bettina, Gombert Michael, Ginzel Sebastian, Borkhardt Arndt
Abstract excerpt
Hereditary defects in several genes have been shown to disturb the normal immune response to EBV and to give rise to severe EBV-induced lymphoproliferation in the recent years. Nevertheless, in many patients, the molecular basis of fatal EBV infection still remains unclear. The Fanconi anemia-associated protein 24 (FAAP24) plays a dual role in DNA repair. By association with FANCM as component of the FA core...
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