| 供货周期： | 一周 |
| 品牌： | Abcam |
| 规格： | 多种抗体规 |
| 货号： | YY-Ab-3507 |
| CAS号： | 详见产品说明 |
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.
产品介绍:中性粒细胞弹性蛋白酶ELANE抗体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.
Database links : UniProtKB/Swiss-Prot: P08246.1
规格：多种抗体规格提供
贮存： 贮存于-20℃
纯化方法：亲和纯化的蛋白
研究领域：细胞生物 免疫学 锌指蛋白
产品应用比例：WB=1:100-500 Elisa=1:500-1000 IP=1:20-100 IHC-P=1:100-500 IHC-F=1:100-500 Flow-Cyt=1/20-1/100
公司生产的中性粒细胞弹性蛋白酶ELANE抗体每个流程都执行严格的检测标准，保证蛋白抗原产品质量，质量稳定，实验效果明显。按理化性质和生物学功能，可将其分为IgM、IgG、IgA、IgE、IgD五类。公司产品经无数次市场验证，若出现质量问题可无条件换货或退货。本司备有上万种产品，所有生化试剂产品都具有价格绝对优势，质量保证，欢迎新老客户垂询！
