Neutrophil elastase [201]

GWB-751BA6

IgM

Human

Cytoplasmic and Secreted

None. Constituents: PBS. pH 7. 4

Cytoplasmic and Secreted Elastases form a subfamily of serine proteases that hydrolyse many proteins in addition to elastin. Neutrophil elastase (elastase 2) hydrolyses proteins within specialized neutrophil lysosomes called azurophil granules as well as proteins of the extracellular matrix following the protein\' s release from activated neutrophils. Elastase 2 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. 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 neutrophil differentiation.

Hydrolysis of proteins including elastin. Preferential cleavage: Val-|-Xaa & gt; Ala-|-Xaa.

Bone marrow cells.

Defects in ELA2 are the cause of autosomal dominant severe congenital neutropenia type 1 (SCN1) [MIM:202700]. Severe congenital neutropenia is a heterogeneous disorder of hematopoiesis characterized by a maturation arrest of granulopoiesis at the level of promyelocytes with peripheral blood absolute neutrophil counts below 0. 5 x 10(9)/l and early onset of severe bacterial infections.

Contains 1 peptidase S1 domain [view classification].