Anti-Neutrophil Elastase (ELANE) Antibody (CAB8953)

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SKU:
CAB8953
Product type:
Antibody
Antibody Type:
Monoclonal Antibody
Reactivity:
Human
Mouse
Host Species:
Rabbit
Isotype:
IgG
Synonyms:
ELA2
GE
HLE
HNE
NE
PMN-E
SCN1
Frequently bought together:

Description

Product Name:Neutrophil Elastase (ELANE) Rabbit mAb
Product Code:CAB8953
Size:20uL, 50uL, 100uL
Synonyms:ELA2, GE, HLE, HNE, NE, PMN-E, SCN1
Applications:WB, IHC, IF
Reactivity:Human, Mouse
Host Species:Rabbit
Immunogen:A synthesized peptide derived from human Neutrophil Elastase (ELANE)
Applications:WB, IHC, IF
Recommended Dilutions:WB 1:500 - 1:2000 IHC 1:50 - 1:200 IF 1:50 - 1:200
Reactivity:Human, Mouse
Positive Samples:THP-1, U-937
Immunogen:A synthesized peptide derived from human Neutrophil Elastase (ELANE)
Purification Method:Affinity purification
Storage:Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 0.05% BSA, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:Email for sequence
Gene ID:1991
Uniprot:P08246
Calculated MW:29kDa
Observed MW:30KDa
UniProt Protein Function: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.
UniProt Protein Details:

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

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: extracellular matrix organization and biogenesis; positive regulation of immune response; 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; positive regulation of MAP kinase activity; extracellular matrix disassembly; collagen catabolic process; negative regulation of inflammatory response; defense response to bacterium; protein catabolic process; response to yeast; leukocyte migration; acute inflammatory response to antigenic stimulus; response to UV; negative regulation of chemokine biosynthetic process

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

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 Code:P08246
NCBI GenInfo Identifier:119292
NCBI Gene ID:1991
NCBI Accession:P08246.1
UniProt Secondary Accession:P08246,P09649, Q6B0D9, Q6LDP5,
UniProt Related Accession:P08246
Molecular Weight:28,518 Da
NCBI Full Name:Neutrophil elastase
NCBI Synonym Full Names:elastase, neutrophil expressed
NCBI Official Symbol:ELANE  
NCBI Official Synonym Symbols:GE; NE; HLE; HNE; ELA2; SCN1; PMN-E  
NCBI Protein Information:neutrophil elastase; elastase-2; medullasin; PMN elastase; leukocyte elastase; elastase 2, neutrophil; human leukocyte elastase; polymorphonuclear elastase; bone marrow serine protease; granulocyte-derived elastase
UniProt Protein Name:Neutrophil elastase
UniProt Synonym Protein Names:Bone marrow serine protease; Elastase-2; Human leukocyte elastase; HLE; Medullasin; PMN elastase
Protein Family:Elastase
UniProt Gene Name:ELANE  
UniProt Entry Name:ELNE_HUMAN

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