<unk> s form a subfamily of serine proteases that hydrolyze many proteins in addition to elastin. Humans have six<unk>  genes that encode the structurally similar proteins<unk>  1, 2, 2A, 2B, 3A, and 3B.<unk>  hydrolyzes proteins within specialized<unk> utrophil lysosomes, called azurophil granules, as well as proteins of the extracellular matrix following the protein's release from activated<unk> utrophils.<unk>  may play a role in degenerative and inflammatory diseases by its proteolysis of collagen-IV and elastin of the extracellular matrix. This protein degrades the outer membrane protein A (OmpA) of E. coli as well as the virulence factors of such bacteria as Shigella, Salmonella and Yersinia. Mutations in this gene are associated with cyclic<unk> utropenia (CyN) and severe congenital<unk> utropenia (SCN). At least 95 disease-causing mutations in this gene have been discovered. This gene is clustered with other serine protease gene family members, azurocidin 1 and proteinase 3 genes, at chromosome 19pter. All 3 genes are expressed coordinately and their protein products are packaged together into azurophil granules during<unk> utrophil differentiation.
Neutrophil elastase
Fibrinolysin
Alpha defensin
Beta defensin
Complement component 5a
Complement component 3
Complement component 4
Intrinsic factor