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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 natural killer and T cells. In humans, mutations in seven genes encoding proteins involved in cytolytic effec...

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Detalles Bibliográficos
Autores principales:	Jessen, Birthe, Kögl, Tamara, Sepulveda, Fernando E., de Saint Basile, Genevieve, Aichele, Peter, Ehl, Stephan
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Frontiers Media S.A. 2013
Materias:	Immunology
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3864253/ https://www.ncbi.nlm.nih.gov/pubmed/24379813 http://dx.doi.org/10.3389/fimmu.2013.00448

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