| 中文名称 | 中性粒细胞弹性蛋白酶ELANE抗体 |
| 别 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; HNE; Human leukocyte elastase; Leukocyte elastase; Leukocyte Elastase Precursor; Medullasin; NE; Neutrophil elastase; Neutrophill elastase; PMN E; PMN Elastase; Polymorphonuclear elastase; SCN1; Serine protease; SERP1. |
| 研究领域 | 细胞生物 信号转导 细胞骨架 细胞外基质 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, (predicted: Rat, Dog, ) |
| 产品应用 | WB=1:500-2000 ELISA=1:500-1000 Flow-Cyt=1μg/Test not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 26kDa |
| 细胞定位 | 细胞浆 细胞外基质 |
| 性 状 | Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human Neutrophil Elastase/ELANE:101-200/267 |
| 亚 型 | IgG |
| 纯化方法 | affinity purified by Protein A |
| 储 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存条件 | Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. |
| PubMed | PubMed |
| 产品介绍 | 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 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 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. SWISS: P08246 Gene ID: 1991 Database links: Entrez Gene: 1991 Human Entrez Gene: 50701 Mouse Omim: 130130 Human SwissProt: P08246 Human SwissProt: Q3UP87 Mouse Unigene: 99863 Human Unigene: 262194 Mouse Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| 产品图片 |  Sample:Spinal cord (Mouse) Lysate at 40 ug Primary: Anti-Neutrophil Elastase (bs-6982R) at 1/300 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 26 kD Observed band size: 48 kD  Sample:HL60(Human) Cell Lysate at 30 ug Primary: Anti-Neutrophil Elastase (bs-6982R) at 1/300 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 26 kD Observed band size: 41 kD  Sample:Cerebrum (Mouse) Lysate at 40 ug Primary: Anti-Neutrophil Elastase (bs-6982R) at 1/300 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 26 kD Observed band size: 31/46 kD  Blank control: A549(blue), the cells were fixed with 2% paraformaldehyde (10 min) and then permeabilized with ice-cold 90% methanol for 30 min on ice..Isotype Control Antibody: Rabbit IgG(orange) ; Secondary Antibody: Goat anti-rabbit IgG-FITC(white blue), Dilution: 1:100 in 1 X PBS containing 0.5% BSA ; Primary Antibody Dilution: 1μl in 100 μL1X PBS containing 0.5% BSA(green). |