LEUKOCYTE ELASTASE

GWB-D078BD

The monoclonal antibody 265-3K1 can be used for immuno assays and Western blotting. The monoclonal antibody 265-3K1 recognizes human leukocyte elastase. Leukocyte elastase. a major serine proteinase in man. is predominantly present in the azurophilic granules of neutrophils and monocytes. Elastase has a broad range of extracellular matrix substrates including elastin. proteoglycans. collagen and fibronectin. The action of elastase is controlled by serine proteinase inhibitors. Elastase. when released during inflammation. is rapidly bound by its two main inhibitors. alpha1-PI and alpha2-macroglobuline to form elastase-inhibitor complexes. In addition mucosa secretions may contain the locally secreted elastase inhibitors elafin/SKALP and SLPI. When secreted at sites of inflammation elastase can cause severe tissue damage. An important role has been suggested for human elastase in various inflammatory disorders. including pulmonary emphysema. sepsis. arthritis. nephritis and certain skin diseases. Elastase induces the production of IL-8 in human bronchial epithelial. a proces that occurs in part through TLR4.

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].