Mixed hematopoietic or T-cell chimerism above a minimal threshold restores perforin-dependent immune regulation in perforin-deficient mice
Open Access
- 10 October 2013
- journal article
- Published by American Society of Hematology in Blood
- Vol. 122 (15) , 2618-2621
Abstract
Key Points. Perforin deficiency causes immune dysregulation and hemophagocytic lymphohistiocytosis, which requires allogeneic HCT for long-term cure.In prf−/− mKeywords
This publication has 17 references indexed in Scilit:
- Perforin deficiency impairs a critical immunoregulatory loop involving murine CD8+ T cells and dendritic cellsBlood, 2013
- Perforin is a critical physiologic regulator of T-cell activationBlood, 2011
- Disruption of MyD88 signaling suppresses hemophagocytic lymphohistiocytosis in miceBlood, 2011
- Reduced-intensity conditioning significantly improves survival of patients with hemophagocytic lymphohistiocytosis undergoing allogeneic hematopoietic cell transplantationBlood, 2010
- Neutralization of IFNγ defeats haemophagocytosis in LCMV‐infected perforin‐ and Rab27a‐deficient miceEMBO Molecular Medicine, 2009
- The use of reduced-intensity stem cell transplantation in haemophagocytic lymphohistiocytosis and Langerhans cell histiocytosisBone Marrow Transplantation, 2008
- Unrelated donor hematopoietic cell transplantation for hemophagocytic lymphohistiocytosisBone Marrow Transplantation, 2008
- Hematopoietic Stem Cell Transplantation in Hemophagocytic Lymphohistiocytosis: A Single-Center Report of 48 PatientsPediatrics, 2006
- Stem cell transplantation with reduced-intensity conditioning for hemophagocytic lymphohistiocytosisBlood, 2006
- An animal model of hemophagocytic lymphohistiocytosis (HLH): CD8+ T cells and interferon gamma are essential for the disorderBlood, 2004