Neutrophil elastase-processing defect in cyclic hematopoietic dogs

Experimental Hematology
Ronghua MengClinton D Lothrop

Abstract

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 aim of this study was to study intracellular sorting/trafficking of NE in CH dogs using antibodies specific to canine NE. Polyclonal and monoclonal antibodies were generated to immunogenic epitopes in the middle (aa85-98) and C-terminal (aa269-282) regions of NE. The antibodies to canine NE were characterized by Western immunoblotting and immunocytochemistry. Antibody ELA85 (antibody to canine NE aa 85-98) specifically recognized mature 28-kD NE. Immunocytochemical analysis using ELA85 and an antibody to myeloperoxidase demonstrated colocalizaton of NE and myeloperoxidase in primary granules of normal dogs. Antibody ELA269 (antibody to canine NE aa 269-282) reacted exclusively with the 33-kD NE presumptive precursor form. Immunocytochemical analysis demonstrated that the NE precursor was not colocalized with myeloperoxidase in the p...Continue Reading

Citations

May 21, 2011·Orphanet Journal of Rare Diseases·Jean DonadieuChristine Bellanné Chantelot
Jun 22, 2013·Blood·Daniel C Link
Jun 12, 2013·Experimental Hematology·Matthew O VallejoClinton D Lothrop
Jan 29, 2013·Hematology/oncology Clinics of North America·Marshall S HorwitzTimothy Tidwell
Jan 31, 2012·The Veterinary Clinics of North America. Small Animal Practice·Shannon Jones Hostetter
Jan 14, 2012·The Journal of Veterinary Medical Science·Keijiro MizukamiOsamu Yamato
Jan 4, 2020·Human Mutation·Marjan HuizingBernadette R Gochuico

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