Subtle differences in CTL cytotoxicity determine susceptibility to hemophagocytic lymphohistiocytosis in mice and humans with Chediak-Higashi syndrome
Jessen, B; Maul-Pavicic, A.; Ufheil, H; Vraetz, T; Enders, Anselm; Lehmberg, K.F.; Langler, A.; Gross-Wieltsch, U.; Bay, A.; Kaya, Z.; Bryceson, Y.T.; Koscielniak, E.; Badawy, S.; Davies, G.; Hufnagel, M.; Schmitt-Graeff, A; Aichele, P; Stadt, U.
Perforin-mediated cytotoxicity is important for controlling viral infections, but also for limiting immune reactions. Failure of this cytotoxic pathway leads to hemophagocytic lymphohistiocytosis (HLH), a life-threatening disorder of uncontrolled T-cell and macrophage activation. We studied susceptibility to HLH in 2 mouse strains (souris and beige J) and a cohort of patients with partial defects in perforin secretion resulting from different mutations in the LYST gene. Although both strains...[Show more]
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