Defective CD107a Surface Expression Heralds Munc13-4 Defect and Discriminates between Genetic Subtypes of Familial Hemophagocytic Lymphohistiocytosis (FHL).
Aricò M, Marcenaro S, Gallo F, Martini S, Santoro A, Griffiths G, Moretta L, Pende D. Defective CD107a Surface Expression Heralds Munc13-4 Defect and Discriminates between Genetic Subtypes of Familial Hemophagocytic Lymphohistiocytosis (FHL). Blood 2006, 108: 1248. DOI: 10.1182/blood.v108.11.1248.1248.Peer-Reviewed Original ResearchFamilial hemophagocytic lymphohistiocytosisNK cellsCD107a expressionFHL3 patientsNatural killerIL-2Genetic subtypesB-EBV cell linesCell surface CD107a expressionSurface expression of CD107aCases of HLHInvestigated natural killerSurface CD107a expressionExpression of CD107aNK cell populationCD56+ cellsNK cell defectPolyclonal NK cellsCentral nervous system alterationsLyse target cellsAnalysis of PBMCsCo-CultureInfiltration of lymphocytesNervous system alterationsMunc13-4 proteinAnalysis 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
This site is protected by hCaptcha and its Privacy Policy and Terms of Service apply