中性粒細胞彈性蛋白酶ELANE抗體

規(guī)格：1mg/1ml

英文名： Neutrophil Elastase

別名： Bone Marrow Serine Protease; ELA 2; ELA2; ELANE; Elastase 2; Elastase-2; Elastase 2 neutrophil; Elastase neutrophil expressed; Elastase-2; ELNE_HUMAN; GE antibody Granulocyte derived elastase; HLE; HN

分子量： 26kDa

儲存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce

克隆類型：Polyclonal

亞型：IgG

純化方法：affinity purified by Protein A

**原：KLH conjugated synthetic peptide derived from human Neutroph

交叉反應(yīng)：Human, Mouse, Rat, Dog,

細胞定位：

中性粒細胞彈性蛋白酶ELANE抗體產(chǎn)品介紹：background: 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]. Function: Modifies the functions of natural killer cells, monocytes and granulocytes. Inhibits C5a-dependent neutrophil enzyme release and 中性粒細胞彈性蛋白酶ELANE抗體chemotaxis. Subunit: Interacts with NOTCH2NL. Tissue Specificity: Bone marrow cells. DISEASE: Defects in ELANE are a cause of cyclic haematopoiesis (CH) [MIM:162800]; 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) [MIM:202700]. SCN1 is a中性粒細胞彈性蛋白酶ELANE抗體 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. Similarity: Belongs to the peptidase S1 family. Elastase subfamily. Contains 1 peptidase S1 domain. Database links: UniProtKB/Swiss-Prot: P08246.1 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

中性粒細胞彈性蛋白酶ELANE抗體產(chǎn)品應(yīng)用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 Flow-Cyt=1:20-500 IF=1:50-200 （石蠟切片需做抗原修復(fù)） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.

研究領(lǐng)域：細胞生物  信號轉(zhuǎn)導(dǎo)  細胞骨架  細胞外基質(zhì)  

儲存條件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.

來源： Rabbit

外觀： Lyophilized or Liquid

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