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Validated All-in-One™ qPCR Primer for ELANE(NM_001972.3) Search again
Product ID:
HQP095164
(click here to view gene annotation page)
Powered by BiozSpecies:
Human
Symbol:
Alias:
ELA2, GE, HLE, HNE, NE, PMN-E, SCN1
Gene Description:
elastase, neutrophil expressed
Target Gene Accession:
NM_001972.3(click here to view gene page)
Estimated Delivery:
Approximately 1-3 weeks, but may vary. Please email sales@genecopoeia.com or call 301-762-0888 to confirm ETA.
Important Note:
By default, qPCR primer pairs are designed to measure the expression level of the splice variant (accession number) you selected for this gene WITHOUT consideration of other possible variants of this gene. If this gene has multiple variants, and you would like to measure the expression levels of one particular variant, multiple variants, or all variants, please contact us for a custom service project at inquiry@genecopoeia.com.
Summary
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 elastase 1, 2, 2A, 2B, 3A, and 3B. Elastase 2 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. Elastase 2 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].
Gene References into function
- Mutant neutrophil elastase induces accelerated apoptosis of promyelocytes, leading to maturation arrest in congenital and cyclic neutropenia.
- UVA light stimulates the production of neutrophil elastase by dermal fibroblasts: a possible contribution to the remodeling of elastotic areas in sun-damaged skin.
- Elastase is mainly responsible for cartilage damage by stimulated human neutrophils, as demonstrated by the use of selective elastase inhibitors.
- Neutrophil elastase-induced overexpression of mucin genes in cultured tumor cells is inhibited by a retinoic acid receptor alpha antagonist.
- degraded scu-PA and also tcu-PA, t-PA and plasmin, resulting in loss of fibrinolytic activity
- Precursor cells containing the ELA2 mutation are selectively lost during myelopoiesis or fail to develop into neutrophils, confirming the pathogenic nature of elastase mutations in humans.
- HNE plays a role in neutrophil response to inflammation
- plasma levels are increased during G-CSF induced hematopoietic stem cell mobilization
- Treatment of human gingival fibroblasts with human leukocyte elastase down-regulated CD40 expression & binding to CD40 ligand. CD40 reduction by direct proteolysis by HLE was seen in skin & lung fibroblasts (not monocytes, macrophages, & dendritic cells)
- Neutrophil elastase plays a role in providing negative feedback to granulopoiesis by direct antagonism of G-CSF.
- Protease entry results in direct cleavage of p65 NF-kappaB in the N-terminal region by PR3 and in the C-terminal region by HNE. PR3 and HNE have specific, fundamental roles in endothelial responses during inflammation.
- REVIEW: role of ela2 germline mutations in inherited neutropenia, gene feedback circuits, signalling genetics, and proposal that neutrophil elastase acts as an inhibitor of myelopoiesis
- adherent PMNs induce a localized, sequential disassembly of endothelial adherens junctions, which is partially mediated by PMN-derived elastase
- Review. Heterozygous mutations in the ELA2 gene have been described in cyclical & congenital neutropenia. A case of paternal mosaicism has provided genetic "proof" of the pathogenicity of such mutations.
- Fibrous and atheromatous plaques but not normal arteries contained NE; NE abounded in the macrophage-rich shoulders of atheromatous plaques with features of vulnerability. Neutrophil elastase and macrophages colocalized in such vulnerable plaques
- Mutations in proto-oncogene GFI1 cause human neutropenia and target ELA2
- Neutrophil elastase up-regulates interleukin-8 via toll-like receptor 4
- Multimeric SFTPD was partially digested by ELA2 dose- and time-dependently with loss of its carbohydrate recognition domain.
- in vitro challenge of neutrophils in asthmatic patients with allergens to which the patients were sensitive elicited a release of elastase by these cells. The in vitro activation of neutrophils was highly allergen specific.
- the effect of kininogen degradation by human neutrophil elastase (HNE) on kinin generation
- elastase overproduction by the leukemic clone can change the growth environment by digesting growth factors, thereby giving advantage to Ph+ hematopoiesis
- NE levels in infected lobes were higher than those in uninvolved lobes, and NE levels were significantly elevated in both, compared with that in control lobes in community acquired pneumonia
- NE-induced degradation of G-CSG and G-CSFR correlates with a reduction in the biologic activity of the cytokine and a decrease in the signaling function of the receptor because of decreased G-CSFR surface expression.
- Since NE is maximally produced in promyelocytes, this protease may play a role in APL pathogenesis by facilitating the leukemogenic potential of PML-RARalpha
- Serine proteinases neutrophil elastase and cathepsin G cooperate for the proteolytic regulation of CD87/urokinase receptor on monocytic cells.
- An important part of the antimicrobial mechanism of neutrophil elastase may be a periplasmic bacteriostatic effect of protease that has translocated across the damaged outer membrane.
- Results indicate that neutrophil elastase activates p38 MAP kinase which upregulates NF-kappaB and AP-1 activities, thus inducing interleukin-8 mRNA expression and protein synthesis.
- the expression of neutropenia in cyclic and severe congenital neutropenia may be either homogeneous or variable according to the type of mutations, suggesting different pathogenetic mechanisms.
- Results demonstrate that human neutrophil elastase is mitogenic for airway smooth muscle cells by increasing cyclin D1 activity through the mitogen-activated protein kinase signaling pathway.
- neutrophil elastase mutations play a role in human hereditary neutropenia, as shown in a dog model [review]
- neutrophil elastase and cathepsin G are inhibited by PAI-1 mutants
- Release of human leukocyte elastase from neutrophils specifically down-regulates the responsiveness of neutrophils to C5a, and this effect may play a role in the down-regulation of acute inflammation.
- elastase released by polymorphonuclear leukocytes trapped within the mural thrombus impairs the spontaneous anchorage of mesenchymal cells to a fibrin matrix
- secreted E coliO78 OmpA can play a role in protection of bacteria from NE by competitive inhibition
- Neutrophil elastase has a role in PML-retinoic acid receptor alpha activities in early myeloid cells
- Neutrophil elastase, MIP-2, and TLR-4 have roles in progression of human sepsis and murine peritonitis
- data suggest mechanisms by which NE mutations cause neutropenia and suggest that abnormal protein trafficking and accelerated apoptosis of differentiating myeloid cells contribute to the severe congenital neutropenia phenotype of the G185R mutation.
- elastase binds to the beta(2)-integrin CD11b and induces a conformational alteration of CD11b
- human AML cells constitutively secrete and express SDF-1-dependent cell-surface elastase, which regulates their migration and proliferation
- Patients with cyclic neutropenia and agranulocytosis showed mutations in this enzyme.
- HNE stimulation of mucin release by human airway epithelial cells involves intracellular activation of PKC, specifically the delta isoform.
- HNE causes protein kinase C (PKC) activation and translocation from cytosol to plasma membrane, required for HNE-induced ROS generation and other responses
- granulocyte-derived elastase is a potentially useful marker for the diagnosis of overt-disseminated intravascular coagulation and as a predictor of organ failure-related outcome
- These data suggest that hypertonic saline upregulates polymorphonuclear neutrophil degranulation via ATP release and positive feedback through P2 and A3 receptors.
- comparison of x-ray structures of alpha1PI complexes with trypsin, PPE, and human neutrophil elastase
- cells expressing mutant HNE exhibited a significant increase in apoptosis associated with up-regulation of master ER chaperone BiP, indicating that disturbance of intracellular trafficking results in activation of the mammalian unfolded protein response
- C-reactive protein degradation products generated by neutrophil elastase promote neutrophil apoptosis
- The Leu92His mutation is near the glycosylation site Asn95. This AA substitution may alter the availability of this Asn residue resulting in abnormal glycosylation of the mutant protein.
- Neutrophil derived elastase (NE) upregulates TGF-beta1 gene expression and release via My88/IRAK/NF-kappaB pathway, possibly through activation of TLR4, and shed light on a potential role of neutrophils in the pathogenesis of asthma.
- The identification of annexin 1 as a substrate for HLE supports the model in which annexin 1 participates in regulating leukocyte emigration into inflamed tissue.
- analysis of human neutrophil protease 3 and elastase active site charge distribution
- NAD(P)H:quinone oxidoreductase 1has an important role as a mediator of neutrophil elastase-regulated oxidant stress and mucin 5AC mucin gene expression.
- The activation of the neutrophil elastase-mediated fibrinolytic pathway may be insufficient to overcome the fibrinolytic shutdown by PAI-1 and may in part explain the poor prognosis of DIC patients associated with systemic inflammation.
- The primary granule proteins elastase (ELA2) and proteinase 3 (PR3) both contain the nonapeptide PR1, which can induce cytotoxic T lymphocyte responses against chronic myeloid leukemia.
- An exceptional genetic phenomenon in which both a cyclic neutropenia patient and an SCN patient each possess two new ELA2 mutations; role in protein mistrafficking as disease mechanism.
- The association of C4890A with myocardial infarction (MI) in Belfast exclusively, and the presumed absence of its functionality, provides little support for a substantial implication of common ELA2 gene variants in overall MI risk.
- Data suggest that polymorphonuclear leukocytes decrease dendritic cell allostimulatory ability via production of elastase leading to a switch of immature DCs into TGF-beta1-secreting cells.
- ELA2 mutations result in the production of misfolded NE protein, activation of the unfolded protein response (UPR), and ultimately apoptosis of granulocytic precursors
- Induction of murine macrophage microbicidal activity by either neutrophils or purified human neutrophil elastase requires TLR4 expression by macrophages.
- Leukocyte elastase is involved in renal injury occurring in the course of primary idiopathic glomerulonephritis, in particular in the proliferative types.
- urinary trypsin inhibitor is insufficiently produced for neutrophil elastase release after cardiac arrest
- study of severe congenital neutropenia patients; there was no significant difference in risk of myelodysplastic syndrome/acute myeloid leukaemia in patients with mutant versus wild-type ELA2
- Genetic evidence suggests that ELA2 mutations act in a dominant, cell-intrinsic fashion to disrupt granulopoiesis. Mutations of ELA2 may cause disease through induction of the unfolded protein response. Review.
- Neutrophil elastase cleaves laminin-332 (laminin-5) generating peptides that are chemotactic for neutrophils.
- Pre-eminent role for neutrophil elastase as the critical protease responsible for PSP-D depletion in inflammatory lung disease
- In an in vitro atherosclerosis model neutrophil infiltration was mediated via IL-8-signalling and accompanied by release of elastase and induction of endothelial cell apoptosis.
- Analysis of the NE proteolytic products of the Shigella virulence factor IpaB shows that NE has specific cleavage sites.
- total amount and the percentage of free elastase activity were significantly higher in juvenile systemic lupus erythematosus when compared with the controls
- This result revealed a negative feedback loop whereby CUX1 shuts down the expression of the protease that cleaves it.
- HLE may be a natural inactivator of MMP-7 which can counteract MMP-7-induced apoptosis resistance.
- CD63 is involved in granule targeting of neutrophil elastase
- elafin is cleaved by its cognate enzyme NE present at excessive concentration in CF sputum, an effect which is promoted by P. aeruginosa infection
- Non-surgical periodontal treatment was effective in reducing the levels of elastase in gingival crevicular fluid from diabetic patients with chronic periodontitis.
- Modulation of endothelial cell tissue factor and tissue factor pathway inhibitor-1 by neutrophil elastase.
- Report changes in plasma level of human leukocyte elastase during leukocytosis from physical effort.
- The hypothesis that an imbalance between neutrophil elastase (HNE) and its inhibitors in blood is related to the development of ARDS, was tested.