Neutrophil Elastase (NE) ELISA Kit for Rabbit

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

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; cellular calcium ion homeostasis; collagen catabolic process; defense response to bacterium; extracellular matrix disassembly; extracellular matrix organization and biogenesis; leukocyte migration; 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; 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