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Neutrophil elastase information


ELANE
Available structures
PDBOrtholog search: PDBe RCSB
Identifiers
AliasesELANE, ELA2, GE, HLE, HNE, NE, PMN-E, SCN1, elastase, neutrophil expressed
External IDsOMIM: 130130; MGI: 2679229; HomoloGene: 20455; GeneCards: ELANE; OMA:ELANE - orthologs
Orthologs
SpeciesHumanMouse
Entrez
Ensembl
UniProt
RefSeq (mRNA)

NM_001972

NM_015779

RefSeq (protein)

NP_001963

NP_056594

Location (UCSC)Chr 19: 0.85 – 0.86 MbChr 10: 79.72 – 79.72 Mb
PubMed search[3][4]
Wikidata
View/Edit HumanView/Edit Mouse

Neutrophil elastase (EC 3.4.21.37, leukocyte elastase, ELANE, ELA2, elastase 2, neutrophil, elaszym, serine elastase, subtype human leukocyte elastase (HLE)) is a serine proteinase in the same family as chymotrypsin and has broad substrate specificity. Neutrophil elastase is secreted by neutrophils during inflammation, and destroys bacteria and host tissue.[5] It also localizes to neutrophil extracellular traps (NETs), via its high affinity for DNA, an unusual property for serine proteases.[6]

As with other serine proteinases it contains a charge relay system composed of the catalytic triad of histidine, aspartate, and serine residues that are dispersed throughout the primary sequence of the polypeptide but that are brought together in the three dimensional conformation of the folded protein. The gene encoding neutrophil elastase, ELA2, consists of five exons. Neutrophil elastase is closely related to other cytotoxic immune serine proteases, such as the granzymes and cathepsin G. It is more distantly related to the digestive CELA1.[6]

The neutrophil form of elastase (EC 3.4.21.37) is 218 amino acids long, with two asparagine-linked carbohydrate chains (see glycosylation). It is present in azurophil granules in the neutrophil cytoplasm. There appear to be two forms of neutrophil elastase, termed IIa and IIb.

  1. ^ a b c ENSG00000197561 GRCh38: Ensembl release 89: ENSG00000277571, ENSG00000197561 – Ensembl, May 2017
  2. ^ a b c GRCm38: Ensembl release 89: ENSMUSG00000020125 – Ensembl, May 2017
  3. ^ "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  4. ^ "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  5. ^ Belaaouaj A, Kim KS, Shapiro SD (August 2000). "Degradation of outer membrane protein A in Escherichia coli killing by neutrophil elastase". Science. 289 (5482): 1185–8. Bibcode:2000Sci...289.1185B. doi:10.1126/science.289.5482.1185. PMID 10947984.
  6. ^ a b Thomas MP, Whangbo J, McCrossan G, Deutsch AJ, Martinod K, Walch M, Lieberman J (June 2014). "Leukocyte protease binding to nucleic acids promotes nuclear localization and cleavage of nucleic acid binding proteins". J. Immunol. 192 (11): 5390–7. doi:10.4049/jimmunol.1303296. PMC 4041364. PMID 24771851.

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Elastase

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connective tissue. The neutrophil form breaks down the Outer membrane protein A (OmpA) of E. coli and other Gram-negative bacteria. Elastase also has the important...

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neutrophilic parasites. In alpha 1-antitrypsin deficiency, the important neutrophil elastase is not adequately inhibited by alpha 1-antitrypsin, leading to excessive...

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dominant and mostly caused by heterozygous mutations in the ELANE gene (neutrophil elastase). Neutropenia can be acute (temporary) or chronic (long lasting)...

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corroborated that NETs contain proteins from azurophilic granules (neutrophil elastase, cathepsin G and myeloperoxidase), specific granules (lactoferrin)...

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SERPINB1

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(ovalbumin related serpins) founded by ovalbumin. MNEI (monocyte/neutrophil elastase inhibitor) is the mouse orthologue of human SerpinB1. SerpinB1 is...

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SLPI

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seminal fluids. SLPI inhibits human leukocyte elastase, human cathepsin G, human trypsin, neutrophil elastase, and mast cell chymase. X-ray crystallography...

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associated with more severe bronchiectasis. Neutrophil elastase, which is an extracellular protein secreted by neutrophils to destroy pathogens as well as host...

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Sivelestat

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research name ONO 5046, marketed as Elaspol) is an inhibitor of human neutrophil elastase. It is used in the treatment of acute respiratory failure and preliminary...

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Biliary atresia

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aflatoxin damaged liver cells and bile duct cells are removed by neutrophil elastase and by involvement of immune system mediators such as CCL-2 or MCP-1...

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Smegma

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of Littré. Smegma contains cathepsin B, lysozymes, chymotrypsin, neutrophil elastase and cytokines, which aid the immune system. According to Wright,...

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Cathepsin G

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related to other immune serine proteases, such as neutrophil elastase and the granzymes. As a neutrophil serine protease, was first identified as degradative...

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Preputial mucosa

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apocrine glands, which secrete cathepsin B, lysozyme, chymotrypsin, neutrophil elastase, and hormones such as androsterone. The first four substances have...

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Antithrombin

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days. However, bonds P3-P4 and P1'-P2' can be rapidly cleaved by neutrophil elastase and the bacterial enzyme thermolysin, respectively, resulting in...

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Serpin

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their active site. Examples include thrombin, trypsin, and human neutrophil elastase. Serpins act as irreversible, suicide inhibitors by trapping an intermediate...

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Elaszym

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Elaszym may refer to one of two enzymes: Pancreatic elastase Neutrophil elastase This disambiguation page lists articles associated with the title Elaszym...

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Ecotin

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immune system, inhibit neutrophil elastase strongly while ecotin from the plant pathogen Pantoea citrea inhibits neutrophil elastase 1000-fold less potently...

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Azurocidin 1

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multifunctional inflammatory mediator. The genes encoding this protein, neutrophil elastase 2, and proteinase 3 are in a cluster located at chromosome 19pter...

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Pulmonary alveolus

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alveoli is broken down by an imbalance between the production of neutrophil elastase (elevated by cigarette smoke) and alpha-1 antitrypsin (the activity...

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Serum protein electrophoresis

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deficiency. This eventually leads to emphysema from unregulated neutrophil elastase activity in the lung tissue. The alpha-1 fraction does not disappear...

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Hair follicle

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damage response involves the proteolysis of type XVII collagen by neutrophil elastase in response to the DNA damage in the hair follicle stem cells. Proteolysis...

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Proteinase 3

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"Characterization of two azurphil granule proteases with active-site homology to neutrophil elastase". The Journal of Biological Chemistry. 265 (4): 2038–41. doi:10...

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Capnocytophaga canimorsus

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to result in decreased superoxide production in neutrophils, as well as declines in neutrophil elastase activity. This results in an increase in predisposition...

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Neuromyelitis optica spectrum disorder

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(non-pathogenic antibody blocker of AQP4-IgG binding), sivelestat (neutrophil elastase inhibitor), and eculizumab (complement inhibitor). There is little...

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Insulin receptor substrate 1

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growth. Tumor infiltrating neutrophils have recently been thought to adjust tumor growth and invasiveness. Neutrophil elastase is shown to degrade IRS-1...

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damage response involves the proteolysis of type XVII collagen by neutrophil elastase in response to DNA damage in hair follicle stem cells. Proteolysis...

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