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  4. ELANE Recombinant Monoclonal Antibody

ELANE Recombinant Monoclonal Antibody

  • 货号：
    CSB-RA200985A0HU
  • 规格：
    ￥1320
  • 图片：
    • IHC image of CSB-RA200985A0HU diluted at 1:100 and staining in paraffin-embedded human spleen tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4℃ overnight. The primary is detected by a Goat anti-rabbit IgG polymer labeled by HRP and visualized using 0.05% DAB.
    • Overlay histogram showing Jurkat cells stained with CSB-RA200985A0HU (red line) at 1:50. The cells were fixed with 70% Ethylalcohol (18h) and then incubated in 10% normal goat serum to block non-specific protein-protein interactions followedby the antibody (1µg/1*106 cells) for 1 h at 4℃.The secondary antibody used was FITC-conjugated goat anti-rabbit IgG (H+L) at 1/200 dilution for 30min at 4℃. Control antibody (green line) was Rabbit IgG (1µg/1*106 cells) used under the same conditions. Acquisition of >10,000 events was performed.
  • 其他：

产品详情

  • 产品描述：

    ELANE encodes neutrophil elastase (NE), a serine protease that is necessary for neutrophil activity and is involved in the inflammatory response to tissue injuries such as sepsis or arthritis. In more than half of SCN cases, the NE gene has been mutated. Though NE is primarily engaged in the antibacterial response, it can have a number of negative consequences, including extracellular matrix breakdown, mucus gland hyperplasia and increased mucus production, ciliary beating rate reduction, and direct injury to the airway epithelium.

    The production of this recombinant ELANE antibody started with identifying and cloning the genes for antibody expression. After the ELANE antibody was cloned into an expression plasmid, the plasmid could be introduced into the mammalian cell to produce the target recombinant antibody. This recombinant ELANE antibody has been validated in ELISA, IHC, FC.

  • Uniprot No.：
    P08246
  • 基因名：
  • 别名：
    Neutrophil elastase (EC 3.4.21.37) (Bone marrow serine protease) (Elastase-2) (Human leukocyte elastase) (HLE) (Medullasin) (PMN elastase), ELANE, ELA2
  • 反应种属：
    Human
  • 免疫原：
    A synthesized peptide derived from human Neutrophil Elastase
  • 免疫原种属：
    Homo sapiens (Human)
  • 标记方式：
    Non-conjugated
  • 克隆类型：
    Monoclonal
  • 抗体亚型：
    Rabbit IgG
  • 纯化方式：
    Affinity-chromatography
  • 克隆号：
    2H12
  • 浓度：
    It differs from different batches. Please contact us to confirm it.
  • 保存缓冲液：
    Rabbit IgG in phosphate buffered saline, pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
  • 产品提供形式：
    Liquid
  • 应用范围：
    ELISA, IHC, FC
  • 推荐稀释比：
    Application Recommended Dilution
    IHC 1:50-1:200
    FC 1:20-1:200
  • Protocols：
  • 储存条件：
    Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
  • 货期：
    Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.

产品评价

靶点详情

  • 功能：
    Modifies the functions of natural killer cells, monocytes and granulocytes. Inhibits C5a-dependent neutrophil enzyme release and chemotaxis. Capable of killing E.coli but not S.aureus in vitro; digests outer membrane protein A (ompA) in E.coli and K.pneumoniae.
  • 基因功能参考文献：
    1. Results found that the neutrophil elastase is up-regulated in both overweight and obese subjects and associated with BMI and markers of cardiovascular disease. PMID: 30056589
    2. Munc134 mediates human neutrophil elastaseinduced airway mucin5AC hypersecretion by interacting with syntaxin2. PMID: 29767240
    3. Neutrophil elastase impaired the EMILIN1-alpha4beta1 integrin interaction by cleaving the gC1q domain in a region crucial for its proper structural conformation, paving the way to better understand NE effects on EMILIN1-cell interaction in pathological context. PMID: 28074935
    4. the effect of elastase on gammadelta T cells was mediated through the protease-activated receptor, PAR1, because the inhibition of this receptor with a specific antagonist, RWJ56110, abrogated the effect of neutrophils on gammadelta T-cell activation. PMID: 28888033
    5. PMN elastase is released by neutrophils in response to Streptococcus pneumoniae pneumolysin. PMID: 27892542
    6. Plasma Neutrophil Elastase and Elafin as Prognostic Biomarker for Acute Respiratory Distress Syndrome. PMID: 28187039
    7. Our results suggest that associated serum levels of proMMP-9, NGAL, proMMP-9/NGAL and NE may reflect the state of systemic inflammation in chronic obstructive pulmonary disease related to cigarette smoking. PMID: 28004483
    8. Using different blood leukocyte and skin resident cell preparations, and recombinant proteins, the s have identified that neutrophil elastase, but not other neutrophil derived proteases, cleaves IL-36Ra into its highly active antagonistic form. PMID: 27101808
    9. Taken together, these observations support that the unusual HNE N-glycosylation, here reported for the first time, is involved in modulating multiple immune functions central to inflammation and infection. PMID: 28630087
    10. results suggest activation of elastase in tendinopathy of the long head of the biceps increases the degradation of elastin to increase the levels of elastase, becoming a vicious cycle to the progressive severity of tendinopathy PMID: 27935111
    11. Coincubation with the inhibitors, particularly MK0339, promoted cell survival and increased formation of mature neutrophils. These studies suggest that cell-permeable inhibitors of neutrophil elastase show promise as novel therapies for ELANE-associated neutropenia. PMID: 28754797
    12. Data suggest that neuropilin-1 (NRP1) functions as a receptor for neutrophil elastase (NE), mediates uptake/absorption of NE, and facilitates cross-presentation of PR1 (a peptide fragment of NE) in breast cancer cells. PMID: 28468826
    13. the impact of NE mutations that are typical of human congenital neutropenia in a murine model of in vitro and in vivo neutrophil differentiation PMID: 27942017
    14. Faecal biomarkers lactoferrin, calprotectin, and PMN-elastase were able to distinguish between ulcerative colitis patients with mucosal healing from clinical remission and mild disease, showed significant correlations with endoscopy, and were predictive of a flare. PMID: 26874351
    15. In conclusion, site-specific CBG N-glycosylation regulates the bioavailability of cortisol in inflamed environments by fine-tuning the RCL proteolysis by endogenous and exogenous elastases. PMID: 27339896
    16. Although circulating nucleosomes and elastase - alpha 1-antitrypsin complexes are at present the best characterized biomarkers for neutrophil extracellular traps formation and neutrophil activation, their lack of association with rs2288904 genotype does not exclude a role for SLC44A2 in neutrophil activation (and possibly NET formation) in Venous Thromboembolism. PMID: 27093231
    17. The levels of both NE and PR3 correlated with absolute neutrophil counts and are reduced in type 1 diabetes mellitus patients. PMID: 26939803
    18. The results of the present study may indicate that NE activates the phosphoinositide-3 kinase/Akt signaling pathway in leukemia cells to inhibit apoptosis and enhance cell proliferation PMID: 27035679
    19. The HNE-TACE signalling pathway has an important role in the process of MUC5AC overexpression in chronic rhinosinusitis. PMID: 26881964
    20. An increased NE: A1 AT ratio is closely associated with liver Inflammation in patients with NASH and could serve as a novel marker to predict NASH in humans PMID: 26444279
    21. Neutrophil elastase activity is deleterious for airway epithelial cell homeostasis. PMID: 26221769
    22. mosaic mutations result in cyclic neutropenia PMID: 25912133
    23. insights into the PPE and HNE inhibition PMID: 26372354
    24. Different ELANE mutants show specific activation of different UPR pathways in congenital neutropenia. PMID: 26567890
    25. Neutrophil elastase was increased in the saliva from COPD patients and additionally distinguished smoking status. PMID: 26018813
    26. Paternal Somatic Mosaicism of a Novel Frameshift Mutation in ELANE Causing Severe Congenital Neutropenia. PMID: 26174650
    27. Sirtinol is a novel HNE inhibitor and may have the potential for clinical application in the treatment of inflammatory lung diseases. PMID: 25666548
    28. Autoprocessing of neutrophil elastase near its active site reduces the efficiency of natural and synthetic elastase inhibitors. PMID: 25857284
    29. analysis of the mechanistic link between neutrophil elastase expression in atherosclerotic plaques and concomitant pro-inflammatory bioactive IL-1beta secretion from endothelial cells PMID: 26269588
    30. In patients with carotid plaques, both circulating and intraplaque polymorphonuclear neutrophils produce IL-8, VEGF and elastase, which are crucial for plaque development and progression. PMID: 25893670
    31. Pathogenesis of ELANE-mutant severe neutropenia revealed by induced pluripotent stem cells. PMID: 26193632
    32. The imbalance between Neutrophil elastase and its inhibitors, such as elafin, presents an important therapeutic target in breast cancer. PMID: 25195861
    33. Neutrophil elastase mainly enhances tissue factor production by monocytes via the phospholipase C/conventional PKC/p38 MAPK pathway, although a novel PKC is also involved. PMID: 25465717
    34. results suggest that elastase-like proteases in the microglial cells surrounding Abeta plaques are responsible for ApoE degradation in the brain. PMID: 25956321
    35. PAR2 and TRPV4 are activated by neutrophil elastase to cause inflammation and pain PMID: 25878251
    36. Three proteins-matrix metalloproteinase-9, neutrophil elastase, and FAM49B-were significantly lower in abundance in samples from women with endometriosis. PMID: 25284053
    37. Sequencing is useful for predicting outcomes of ELANE-associated neutropenia. PMID: 25427142
    38. Over-expressed NE in K562 leukemia cells promotes cell proliferation, inhibits apoptosis and block cell cycle in S phase. PMID: 25652853
    39. Suggest a potential role of ELANE gene polymorphisms as therapy modulators in childhood bronchiectasis. PMID: 24816969
    40. An interactive network of elastase, secretases, and PAR-2 protein regulates CXCR1 receptor surface expression on neutrophils PMID: 24914212
    41. Circulating protein levels and enzymatic activity of ELANE are markedly elevated in patients with type 1 diabetes mellitus, and are correlated with increased autoantibodies against beta-cell antigens. PMID: 25092677
    42. The present findings highlight the peculiar activity of PMN elastase in disabling EMILIN1 suppressor function. PMID: 24513040
    43. It reports the synthesis and enzymatic studies on a new proteinase 3 intermolecular quenched substrate with enhanced selectivity over neutrophil elastase. PMID: 23911525
    44. The leukocyte elastase gene is substituted at the 597th base (G to A) in intron 4 of severe congenital neutropenia. PMID: 24616599
    45. Human sex hormone-binding globulin mediates the targeted release of cortisol at sites of inflammation through cleavage of its reactive center loop by neutrophil elastase. PMID: 24848868
    46. neutrophil elastase reflects the severity and progression of acute pancreatitis. PMID: 23819644
    47. ELANE mutations disrupt translation initiation and are associated with neutropenia; they produce novel neutrophil elastase isoforms PMID: 24184683
    48. SPLUNC1 degradation by neutrophil elastase may increase airway susceptibility to bacterial infections. PMID: 23741370
    49. Neutrophil elastase and proteinase-3 trigger G protein-biased signaling through proteinase-activated receptor-1 (PAR1). PMID: 24052258
    50. Specific ELANE mutations have limited predictive value for leukemogenesis; the risk for leukemia was correlated with disease severity rather than with occurrence of an ELANE mutation. PMID: 23463630

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  • 相关疾病：
    Cyclic haematopoiesis (CH); Neutropenia, severe congenital 1, autosomal dominant (SCN1)
  • 亚细胞定位：
    Cytoplasmic vesicle, phagosome.
  • 蛋白家族：
    Peptidase S1 family, Elastase subfamily
  • 组织特异性：
    Bone marrow cells. Neutrophil.
  • 数据库链接：

    HGNC: 3309

    OMIM: 130130

    KEGG: hsa:1991

    STRING: 9606.ENSP00000263621

    UniGene: Hs.99863