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中性粒细胞弹性蛋白酶ELANE抗体
  • 产品货号:
    BN41958R
  • 中文名称:
    中性粒细胞弹性蛋白酶ELANE抗体
  • 英文名称:
    Rabbit anti-Neutrophil Elastase Polyclonal antibody
  • 货号

    产品规格

    售价

    备注

  • BN41958R-50ul

    50ul

    ¥1486.00

    交叉反应:Human,Mouse,Rat(predicted:Dog) 推荐应用:WB,Flow-Cyt,ELISA

  • BN41958R-100ul

    100ul

    ¥2360.00

    交叉反应:Human,Mouse,Rat(predicted:Dog) 推荐应用:WB,Flow-Cyt,ELISA

  • BN41958R-200ul

    200ul

    ¥3490.00

    交叉反应:Human,Mouse,Rat(predicted:Dog) 推荐应用:WB,Flow-Cyt,ELISA

英文名称Neutrophil Elastase
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=1:5000-10000 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.
PubMedPubMed
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.






















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