Graded defects in cytotoxicity determine severity of hemophagocytic lymphohistiocytosis in humans and mice

Graded defects in cytotoxicity determine severity of hemophagocytic lymphohistiocytosis in humans and mice

Primary hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease of hyperinflammation resulting from immune dysregulation due to inherited defects in the cytolytic machinery of NK and T cells. In humans, mutations in seven genes encoding proteins involved in cytolytic effector function...

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Bibliographic Details
Main Authors: Birthe eJessen, Tamara eKögl, Fernando E. Sepulveda, Genevieve ede Saint Basile, Peter eAichele, Stephan eEhl
Format: Article
Language:English
Published: Frontiers Media S.A. 2013-12-01
Series:Frontiers in Immunology
Subjects:
Inflammation
Cytotoxicity
CTL
Hemophagocytic lymphohistiocytosis (HLH)
virus persistence
antigen persistence
Online Access:http://journal.frontiersin.org/Journal/10.3389/fimmu.2013.00448/full

Internet

http://journal.frontiersin.org/Journal/10.3389/fimmu.2013.00448/full

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