ELANE Recombinant Monoclonal Antibody

說明書

中文名稱：

ELANE重組抗體
貨號：

CSB-RA200985A0HU
規格：

￥1320
圖片：
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：

產品詳情
產品評價
相關產品
靶點詳情

產品詳情

產品描述：

ELANE Recombinant Monoclonal Antibody（Code: CSB-RA200984A0HU）是一款針對人源中性粒細胞彈性蛋白酶（ELANE）的高特異性抗體，適用于ELISA、免疫組化（IHC）和流式細胞術（FC）等多種檢測平臺。ELANE是一種絲氨酸蛋白酶，主要由中性粒細胞分泌，在固有免疫防御中發揮關鍵作用，通過降解病原體相關蛋白參與炎癥反應，其基因突變與周期性中性粒細胞減少癥等遺傳性疾病密切相關。該抗體經嚴格驗證，在IHC實驗中可清晰定位組織樣本中的ELANE蛋白表達，推薦稀釋比例為1:50-1:200；在流式細胞分析中能有效檢測細胞表面或胞內ELANE信號，建議使用濃度為1:20-1:200。其高批次間一致性及低交叉反應性確保了實驗結果的可靠性，適用于研究中性粒細胞功能、炎癥調控機制及相關疾病的分子病理學探索，為感染免疫、自身免疫性疾病及血液系統異常等領域的體外研究提供重要工具。
Uniprot No.：

P08246
基因名：

ELANE
別名：

Neutrophil elastase (EC 3.4.21.37) (Bone marrow serine protease) (Elastase-2) (Human leukocyte elastase) (HLE) (Medullasin) (PMN elastase), ELANE, ELA2
反應種屬：

Human
免疫原：

A synthesized peptide derived from human Neutrophil Elastase
免疫原種屬：

Homo sapiens (Human)
標記方式：

Non-conjugated
克隆類型：

Monoclonal
抗體亞型：

Rabbit IgG
純化方式：

Affinity-chromatography
克隆號：

2H12
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

Rabbit IgG in 10mM phosphate buffered saline , pH 7.4, 150mM sodium chloride, 0.05% BSA, 0.02% sodium azide and 50% glycerol.
產品提供形式：

Liquid
應用范圍：

ELISA, IHC, FC

推薦稀釋比：

Application	Recommended Dilution
IHC	1:50-1:200
FC	1:20-1:200

Protocols：

ELISA Protocol
Immunohistochemistry (IHC) Protocol
Flow cytometry (FC) Protocol
儲存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
貨期：

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.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

產品評價

靶點詳情

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.
基因功能參考文獻：
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 authors 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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相關疾病：

Cyclic haematopoiesis (CH); Neutropenia, severe congenital 1, autosomal dominant (SCN1)
亞細胞定位：

Cytoplasmic vesicle, phagosome.
蛋白家族：

Peptidase S1 family, Elastase subfamily
組織特異性：

Bone marrow cells. Neutrophil.
數據庫鏈接：

HGNC: 3309

OMIM: 130130

KEGG: hsa:1991

STRING: 9606.ENSP00000263621

UniGene: Hs.99863

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