Neutrophil elastase Antibody

Background:

Elastases form a subfamily of serine proteases thathydrolyze many proteins in addition to elastin. Humans have sixelastase genes which encode the structurally similar proteins. Theproduct of this gene hydrolyzes proteins within specializedneutrophil lysosomes, called azurophil granules, as well asproteins of the extracellular matrix following the protein'srelease from activated neutrophils. The enzyme may play a role indegenerative and inflammatory diseases by its proteolysis ofcollagen-IV and elastin of the extracellular matrix. This proteindegrades the outer membrane protein A (OmpA) of E. coli as well asthe virulence factors of such bacteria as Shigella, Salmonella andYersinia. Mutations in this gene are associated with cyclicneutropenia and severe congenital neutropenia (SCN). This gene isclustered with other serine protease gene family members,azurocidin 1 and proteinase 3 genes, at chromosome 19pter. All 3genes are expressed coordinately and their protein products arepackaged together into azurophil granules during neutrophildifferentiation.