catalog number :
MBS721788
products type :
ELISA Kit
products full name :
Guinea pig Neutrophil Elastase ELISA Kit
products short name :
Neutrophil Elastase
other names :
neutrophil elastase preproprotein; Neutrophil elastase; neutrophil elastase; elastase-2; medullasin; PMN elastase; leukocyte elastase; elastase 2, neutrophil; human leukocyte elastase; polymorphonuclear elastase; bone marrow serine protease; granulocyte-derived elastase; elastase, neutrophil expressed; Bone marrow serine protease; Elastase-2; Human leukocyte elastase; HLE; Medullasin; PMN elastase
other gene names :
ELANE; ELANE; GE; NE; HLE; HNE; ELA2; SCN1; PMN-E; ELA2; HLE
uniprot entry name :
ELNE_HUMAN
storage stability :
Store all reagents at 2-8 degree C.
other info1 :
Assay Type: Competitive
other info2 :
Highest Standards: 10ng/mL. Concentration of Standards: Concentration of Standards: 0,0.5,1.0,2.5,5.0,10ng/mL
products categories :
Microbiology
ncbi acc num :
NP_001963.1
ncbi gb acc num :
NM_001972.2
ncbi mol weight :
28,518 Da
ncbi pathways :
Activation Of Matrix Metalloproteinases Pathway (576264); C-MYB Transcription Factor Network Pathway (138073); Collagen Degradation Pathway (730309); Degradation Of The Extracellular Matrix Pathway (576263); Extracellular Matrix Organization Pathway (576262); Systemic Lupus Erythematosus Pathway (83122); Systemic Lupus Erythematosus Pathway (534); Transcriptional Misregulation In Cancer Pathway (523016); Transcriptional Misregulation In Cancer Pathway (522987); Amb2 Integrin Signaling Pathway (137945)
ncbi summary :
Elastases form a subfamily of serine proteases that hydrolyze many proteins in addition to elastin. Humans have six elastase genes which encode the structurally similar proteins. The product of this gene hydrolyzes proteins within specialized neutrophil lysosomes, called azurophil granules, as well as proteins of the extracellular matrix following the protein's release from activated neutrophils. The enzyme 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 neutropenia and severe congenital neutropenia (SCN). 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. [provided by RefSeq, May 2009]
uniprot summary :
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; Protease; Cell surface; EC 3.4.21.37; Motility/polarity/chemotaxis. Chromosomal Location of Human Ortholog: 19p13.3. Cellular Component: cell surface; transcriptional repressor complex; cytoplasm; extracellular region; secretory granule. Molecular Function: heparin binding; peptidase activity; protein binding; protease binding; cytokine binding; serine-type endopeptidase activity; endopeptidase activity. Biological Process: positive regulation of immune response; extracellular matrix organization and biogenesis; positive regulation of smooth muscle cell proliferation; negative regulation of interleukin-8 biosynthetic process; positive regulation of interleukin-8 biosynthetic process; response to lipopolysaccharide; negative regulation of chemotaxis; negative regulation of transcription from RNA polymerase II promoter; proteolysis; phagocytosis; cellular calcium ion homeostasis; extracellular matrix disassembly; collagen catabolic process; positive regulation of MAP kinase activity; negative regulation of inflammatory response; defense response to bacterium; response to yeast; protein catabolic process; leukocyte migration; negative regulation of chemokine biosynthetic process; response to UV; acute inflammatory response to antigenic stimulus. Disease: Cyclic Neutropenia; Neutropenia, Severe Congenital, 1, Autosomal Dominant