Neutrophil Elastase (E9C9L) R mAb (Alexa Fluor 647 Conj

Neutrophil elastase is a hematopoietic serine protease that belongs to the chymotrypsin superfamily and plays a critical role in the innate immune function of mature neutrophils and monocytes (1,2). Neutrophil elastase is actively synthesized as an inactive zymogen in myelocytic precursor cells of the bone marrow, which then undergoes activation by limited proteolysis and sorting to primary (azurophil) storage granules of mature neutrophil granulocytes for regulated release (3,4). Research studies have shown that neutrophils play a significant role in mediating the inflammatory response through the release of neutrophil elastase, which activates pro-inflammatory cytokines and degrades components of the extracellular matrix and Gram-negative bacteria (5). Mutations in the gene encoding neutrophil elastase, ELA2, have been implicated in hematological diseases such as cyclic and severe congenital neutropenia, which is characterized by defects in promyelocyte maturation (6,7). 1.Belaaouaj, A. et al. (2000) Science 289, 1185-8. 2.Tkalcevic, J. et al. (2000) Immunity 12, 201-10. 3.Gullberg, U. et al. (1999) J Immunol Methods 232, 201-10. 4.Takahashi, H. et al. (1988) J Biol Chem 263, 2543-7. 5.Adkison, A.M. et al. (2002) J Clin Invest 109, 363-71. 6.Horwitz, M. et al. (1999) Nat Genet 23, 433-6. 7.Bellanné-Chantelot, C. et al. (2004) Blood 103, 4119-25.