Analysis of natural killer–cell function in familial hemophagocytic lymphohistiocytosis (FHL): defective CD107a surface expression heralds Munc13-4 defect and discriminates between genetic subtypes of the disease
Marcenaro S, Gallo F, Martini S, Santoro A, Griffiths G, Aricó M, Moretta L, Pende D. Analysis of natural killer–cell function in familial hemophagocytic lymphohistiocytosis (FHL): defective CD107a surface expression heralds Munc13-4 defect and discriminates between genetic subtypes of the disease. Blood 2006, 108: 2316-2323. PMID: 16778144, DOI: 10.1182/blood-2006-04-015693.Peer-Reviewed Original ResearchConceptsFamilial hemophagocytic lymphohistiocytosisHealthy control subjectsPerforin-deficient NK cellsNK cellsControl subjectsNatural killerHemophagocytic lymphohistiocytosisCytokine productionB-EBV cell linesNatural killer-cell functionSurface CD107a expressionNK cell defectDendritic cell targetingLyse target cellsCD107a surface expressionIdentification of patientsSusceptible tumor targetsMunc13-4 proteinCD107a stainingCD107a expressionB EBVTumor targetingIL-2Genetic subtypesTriggering receptor
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