ELANE; HLE; HNE; NE; PMN-E; Neutrophil Elastase; Medullasin; Polymorphonuclear Leukocyte Elastase; Bone Marrow Serine Protease; Human Leukocyte Elastase; PMN Elastase

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit

200ug/ml (lot specific)

Manufactured in an ISO 9001:2008 and ISO 13485:2003 Certified Laboratory.

Manufactured in a lab with traceable raw materials. Bulk orders can typically be prepared to the customera??s specifications, please inquire.

Small volumes of anti-ELA2 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Western Blot (WB), Immunohistochemistry (IHC)

28,518 Da

elastase, neutrophil expressed

neutrophil elastase

Neutrophil elastase

ELA2; HLE??[Similar Products]

Elastases form a subfamily of serine proteases that hydrolyze many proteins in addition to elastin. Humans have six elastase genes which encode structurally similar proteins. The encoded preproprotein is proteolytically processed to generate the active protease. Following activation, this protease hydrolyzes proteins within specialized neutrophil lysosomes, called azurophil granules, as well as proteins of the extracellular matrix. The enzyme may play a role in degenerative and inflammatory diseases through proteolysis of collagen-IV and elastin. This protein also 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 neutropenia and severe congenital neutropenia (SCN). This gene is present in a gene cluster on chromosome 19. [provided by RefSeq, Jan 2016]

ELANE: Modifies the functions of natural killer cells, monocytes and granulocytes. Inhibits C5a-dependent neutrophil enzyme release and chemotaxis. Defects in ELANE are a cause of cyclic haematopoiesis (CH); also known as cyclic neutropenia. CH is an autosomal dominant disease in which blood-cell production from the bone marrow oscillates with 21-day periodicity. Circulating neutrophils vary between almost normal numbers and zero. During intervals of neutropenia, affected individuals are at risk for opportunistic infection. Monocytes, platelets, lymphocytes and reticulocytes also cycle with the same frequency. Defects in ELANE are the cause of neutropenia severe congenital autosomal dominant type 1 (SCN1). SCN1 is a 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. Belongs to the peptidase S1 family. Elastase subfamily.

Protein type: Cell cycle regulation; Cell surface; EC 3.4.21.37; Motility/polarity/chemotaxis; Protease

Chromosomal Location of Human Ortholog: 19p13.3

Cellular Component: cell surface; cytoplasm; extracellular region; extracellular space; secretory granule; transcriptional repressor complex

Molecular Function: cytokine binding; endopeptidase activity; heparin binding; peptidase activity; protease binding; protein binding; serine-type endopeptidase activity

Biological Process: acute inflammatory response to antigenic stimulus; antimicrobial humoral response; biosynthetic process of antibacterial peptides active against Gram-negative bacteria; cellular calcium ion homeostasis; defense response to bacterium; extracellular matrix disassembly; leukocyte migration involved in inflammatory response; negative regulation of chemokine biosynthetic process; negative regulation of chemotaxis; negative regulation of inflammatory response; negative regulation of interleukin-8 biosynthetic process; negative regulation of transcription from RNA polymerase II promoter; neutrophil degranulation; phagocytosis; positive regulation of immune response; positive regulation of interleukin-8 biosynthetic process; positive regulation of MAP kinase activity; positive regulation of smooth muscle cell proliferation; protein catabolic process; proteolysis; response to lipopolysaccharide; response to UV; response to yeast

Disease: Cyclic Neutropenia; Neutropenia, Severe Congenital, 1, Autosomal Dominant

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