Severe congenital neutropenias are a heterogeneous group of rare haematological
diseases characterized by impaired maturation of neutrophil granulocytes. Patients with …

Häger M, Cowland JB, Borregaard N (University of Copenhagen, Copenhagen, Denmark).
Neutrophil granules in health and disease (Review). J Intern Med 2010; 268: 25–34 …

Autosomal recessive severe congenital neutropenia (SCN) constitutes a primary
immunodeficiency syndrome associated with increased apoptosis in myeloid cells,, yet the …

Background The main features of severe congenital neutropenia are the onset of severe
bacterial infections early in life, a paucity of mature neutrophils, and an increased risk of …

We identified nicotinamide phosphoribosyltransferase (NAMPT), also known as pre-B cell
colony enhancing factor (PBEF), as an essential enzyme mediating granulocyte colony …

The diversity of mutations and clinical outcomes for ELANE-a... : Current Opinion in
Hematology The diversity of mutations and clinical outcomes for ELANE-associated …

Point mutations in the gene for the granulocyte colony-stimulating factor (G-CSF) receptor
CSF3R have been implicated in the progression of severe congenital neutropenia (CN) to …

Severe congenital neutropenia (CN) is a preleukemic bone marrow failure syndrome with a
20% risk of evolving into leukemia or myelodysplastic syndrome (MDS). Patterns of …

Interleukin-17 (IL-17) is a proinflammatory cytokine produced by adaptive CD4+ T helper
cells and innate lymphocytes, such as γδ-T cells and TCRβ+“natural” Th17 cells. IL-17 …

We demonstrate here that lymphoid enhancer-binding factor 1 (LEF-1) mediates the
proliferation, survival and differentiation of granulocyte progenitor cells. We initially …