Article
Neutrophil elastase-processing defect in cyclic hematopoietic dogs.
Experimental hematology - 1 Feb 2010
Meng Ronghua, Bridgman Roger, Toivio-Kinnucan Maria, Niemeyer Glenn P, Vernau William, Hock Tommy, Lothrop Clinton D
Abstract excerpt
OBJECTIVE: Canine cyclic hematopoiesis (CH), a model of human cyclic neutropenia and severe congenital neutropenia, is characterized by a periodic reduced neutrophil count and decreased neutrophil elastase (NE) enzymatic activity. Canine CH is caused by a mutation of AP3B1 encoding the beta3A subunit of adaptor protein complex-3 (AP-3). It has been proposed that trafficking of elastase is affected by AP-3. The...
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