ELANE Antibody

Code CSB-RA200985A0HU
Size US$350Purchase it in Cusabio online store
(only available for customers from the US)
Image
  • 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°C 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*106cells) for 1 h at 4°C.The secondary antibody used was FITC-conjugated goat anti-rabbit IgG (H+L) at 1/200 dilution for 30min at 4°C. Control antibody (green line) was Rabbit IgG (1µg/1*106cells) used under the same conditions. Acquisition of >10,000 events was performed.
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Product Details

Uniprot No. P08246
Target Names ELANE
Alternative Names Neutrophil elastase (EC 3.4.21.37) (Bone marrow serine protease) (Elastase-2) (Human leukocyte elastase) (HLE) (Medullasin) (PMN elastase), ELANE, ELA2
Species Reactivity Human
Immunogen A synthesized peptide derived from human Neutrophil Elastase
Immunogen Species Homo sapiens (Human)
Conjugate Non-conjugated
Clonality Monoclonal
Isotype Rabbit IgG
Clone No. 2H12
Purification Method Affinity-chromatography
Concentration It differs from different batches. Please contact us to confirm it.
Buffer Rabbit IgG in phosphate buffered saline, pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
Form Liquid
Tested Applications ELISA, IHC, FC
Recommended Dilution
Application Recommended Dilution
IHC 1:50-1:200
FC 1:20-1:200
Protocols ELISA Protocol
Immunohistochemistry (IHC) Protocol
Flow Cytometry (FC) Protocol
Troubleshooting and FAQs Antibody FAQs
Storage Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Lead Time 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.

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Target Data

Function Modifies the functions of natural killer cells, monocytes and granulocytes. Inhibits C5a-dependent neutrophil enzyme release and chemotaxis.
Gene References into Functions
  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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Involvement in disease Cyclic haematopoiesis (CH); Neutropenia, severe congenital 1, autosomal dominant (SCN1)
Protein Families Peptidase S1 family, Elastase subfamily
Tissue Specificity Bone marrow cells.
Database Links

HGNC: 3309

OMIM: 130130

KEGG: hsa:1991

STRING: 9606.ENSP00000263621

UniGene: Hs.99863

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