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Letter

Nature Genetics 35, 90–96 (1 September 2003) | doi:10.1038/ng1224

Mutations associated with neutropenia in dogs and humans disrupt intracellular transport of neutrophil elastase

Kathleen F Benson , Feng-Qian Li , Richard E Person , Dalila Albani , Zhijun Duan , Jeremy Wechsler , Kimberly Meade-White , Kayleen Williams , Gregory M Acland , Glenn Niemeyer , Clinton D Lothrop & Marshall Horwitz

Cyclic hematopoiesis is a stem cell disease in which the number of neutrophils and other blood cells oscillates in weekly phases. Autosomal dominant mutations of ELA2, encoding the protease neutrophil elastase, found in lysosome-like granules, cause cyclic hematopoiesis and most cases of the pre-leukemic disorder severe congenital neutropenia (SCN; ref.