A lack of secretory leukocyte protease inhibitor (SLPI) causes defects in granulocytic differentiation

We identified diminished levels of the natural inhibitor of neutrophil elastase (NE), secretory leukocyte protease inhibitor (SLPI), in myeloid cells and plasma of patients with severe congenital neutropenia (CN). We further found that downregulation of SLPI in CD34+ bone marrow (BM) hematopoietic