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Letter

Nature Medicine 12, 1191–1197 (1 November 2006) | doi:10.1038/nm1484

LEF-1 is crucial for neutrophil granulocytopoiesis and its expression is severely reduced in congenital neutropenia

Julia Skokowa , Gunnar Cario , Murat Uenalan , Axel Schambach , Manuela Germeshausen , Karin Battmer , Cornelia Zeidler , Ulrich Lehmann , Matthias Eder , Christopher Baum , Rudolf Grosschedl , Martin Stanulla , Michaela Scherr & Karl Welte

We demonstrate here that lymphoid enhancer-binding factor 1 (LEF-1) mediates the proliferation, survival and differentiation of granulocyte progenitor cells. We initially documented the importance of this transcription factor in the bone marrow of individuals with severe congenital neutropenia (CN) with a 'differentiation block' at the promyelocytic stage of myelopoiesis.