Antibody Name:	Anti-ELANE Antibody
Antibody SKU:	CAB13015
Antibody Size:	20uL, 50uL, 100uL
Application:	WB IF
Reactivity:	Human, Mouse, Rat
Host Species:	Rabbit
Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 30-267 of human ELANE (NP_001963.1).

Application:	WB IF
Recommended Dilution:	WB 1:500 - 1:2000 IF 1:50 - 1:200
Reactivity:	Human, Mouse, Rat
Positive Samples:	Rat spleen

Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 30-267 of human ELANE (NP_001963.1).
Purification Method:	Affinity purification
Storage Buffer:	Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:	IgG
Sequence:	IVGG RRAR PHAW PFMV SLQL RGGH FCGA TLIA PNFV MSAA HCVA NVNV RAVR VVLG AHNL SRRE PTRQ VFAV QRIF ENGY DPVN LLND IVIL QLNG SATI NANV QVAQ LPAQ GRRL GNGV QCLA MGWG LLGR NRGI ASVL QELN VTVV TSLC RRSN VCTL VRGR QAGV CFGD SGSP LVCN GLIH GIAS FVRG GCAS GLYP DAFA PVAQ FVNW IDSI IQRS EDNP CPHP RDPD PASR TH
Gene ID:	1991
Uniprot:	P08246
Cellular Location:
Calculated MW:	28kDa
Observed MW:	30KDa

Synonyms:

ELANE, ELA2, GE, HLE, HNE, NE, PMN-E, SCN1

Background:

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.

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

	Western blot analysis of extracts of Rat spleen, using ELANE antibody (CAB13015) at 1:500 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Enhanced Kit (RM00021). Exposure time: 180s.
	Immunofluorescence analysis of rat bone marrow using ELANE Rabbit pAb (CAB13015) at dilution of 1:100 (40x lens). Blue: DAPI for nuclear staining.
	Immunofluorescence analysis of mouse bone marrow using ELANE Rabbit pAb (CAB13015) at dilution of 1:100 (40x lens). Blue: DAPI for nuclear staining.