ABSTRACT
Human cyclic neutropenia, also referred to as cyclic hematopoiesis, is a rare disease characterized by periodic fluctuations in blood cell production by the bone marrow and a corresponding recurrent severe neutropenia every 19-21 days. This results in bacterial infections and shortened life expectancy. Platelets, monocytes, lymphocytes, and reticulocytes cycle with the same periodicity. It has been determined that the neutrophil elastase (NE) gene is mutated in all cases of human cyclic hematopoiesis. Currently, the only animal model for this disease is the grey collie dog, in which there is a strikingly similar periodic neutropenia every 12-14 days. Towards the validation of this animal model, we have cloned and sequenced the canine NE cDNA from a normal dog.
