3.4.21.37: leukocyte elastase
This is an abbreviated version!
For detailed information about leukocyte elastase, go to the full flat file.
Word Map on EC 3.4.21.37
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3.4.21.37
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pulmonary
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cathepsin
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airway
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pancreatic
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lavage
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myeloperoxidase
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hne
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emphysema
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fibrosis
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cystic
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bronchoalveolar
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granule
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elastin
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sputum
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bronchial
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thrombin
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chymotrypsin
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coagulation
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1-antitrypsin
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neutropenia
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interleukin-8
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1-proteinase
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degranulation
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c-reactive
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cardiopulmonary
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elastolytic
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antiprotease
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serpins
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mucus
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slpi
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alpha1-antitrypsin
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azurophilic
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aa
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neutrophil-derived
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elastases
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2-macroglobulin
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bronchiectasis
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fmlp
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elastase-induced
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netosis
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antineutrophil
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proteinase-3
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thrombin-antithrombin
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ulinastatin
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crevicular
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neutrophil-mediated
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ne-induced
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beta-thromboglobulin
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drug development
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wegener
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2-antiplasmin
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diagnostics
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medicine
- 3.4.21.37
- pulmonary
- cathepsin
- airway
- pancreatic
-
lavage
- myeloperoxidase
- hne
- emphysema
- fibrosis
-
cystic
-
bronchoalveolar
- granule
- elastin
- sputum
- bronchial
- thrombin
- chymotrypsin
- coagulation
-
1-antitrypsin
- neutropenia
- interleukin-8
-
1-proteinase
-
degranulation
-
c-reactive
-
cardiopulmonary
-
elastolytic
-
antiprotease
- serpins
- mucus
- slpi
- alpha1-antitrypsin
-
azurophilic
- aa
-
neutrophil-derived
- elastases
-
2-macroglobulin
- bronchiectasis
- fmlp
-
elastase-induced
-
netosis
-
antineutrophil
- proteinase-3
-
thrombin-antithrombin
- ulinastatin
-
crevicular
-
neutrophil-mediated
-
ne-induced
-
beta-thromboglobulin
- drug development
-
wegener
-
2-antiplasmin
- diagnostics
- medicine
Reaction
Hydrolysis of proteins, including elastin. Preferential cleavage Val-/- > Ala-/- =
Synonyms
bone marrow serine protease, EC 3.4.21.11, EC 3.4.4.7, ELA2, Elane, elastase, elaszym, granulocyte elastase, H2A specific protease, H2Asp, HLE, HNE, leukocyte elastase, lysosomal elastase, N-elastase, NE, neutrophil elastase, neutrophil-elastase, neutrophil-released elastase, PMN elastase, PMN-elastase, PMNE, polymorphonuclear leukocyte elastase, polymorphonuclear neutrophil elastase, serine elastase
ECTree
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General Information
General Information on EC 3.4.21.37 - leukocyte elastase
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malfunction
physiological function
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canine cyclic hematopoiesis, caused by a mutation of AP3B1 encoding the b3A subunit of adaptor protein complex L3, AP-3, and a model of human cyclic neutropenia and severe congenital neutropenia, is characterized by a periodic reduced neutrophil count and decreased neutrophil elastase enzymatic activity. Intracellular sorting/trafficking of neutrophil elastase in cyclic hematopoiesis dogs, neutrophils from cyclic hematopoiesis dogs accumulate large amounts of higher molecular weight elastase precursors compared to normal dogs, overview
malfunction
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ELA2 mRNA expression in myeloid progenitors and plasma protein levels of neutrophil elastase are markedly reduced in patients with congenital neutropenia harboring mutations in either ELA2 or HAX-1 genes
malfunction
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esophagectomy with elective lymphadenectomy may induce excessive release of neutrophil elastase, which then promotes vascular permeability and an excessive water shift from the intravascular space to the peripheral compartment. Body fluid imbalance after esophagectomy often leads to circular instability, a decrease of urine output, and a delay in the shift to a diuretic state. Treatment with inhibitor sivelastat shortens the shift to a diuretic state and leads to a shorter operation time, the incidence of delayed respiratory dysfunction is also significantly lower
malfunction
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excessive neutrophil elastase activity and altered vascular endothelial growth factor signaling are independently in the development and progression of pulmonary emphysema
malfunction
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excessive neutrophil elastase activity and altered vascular endothelial growth factor signaling are independently in the development and progression of pulmonary emphysema
malfunction
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excessive neutrophil elastase activity and altered vascular endothelial growth factor signaling are independently in the development and progression of pulmonary emphysema
malfunction
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granulocyte elastase is involved in preterm and is increased in cervical secretion before gestation, urinary trypsin inhibitor reduces the risk of preterm delivery and improved neonatal outcomes
malfunction
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imbalance between neutrophil elastase and alpha-1-antitrypsin leads to emphysema in smokers as well as in patients with inherited alpha-1-antitrypsin deficiency, overview
malfunction
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increased neutrophil elastase activity and oxidative stress in the lung can lead to alpha-1-antitrypsin deficiency, A1AT deficiency. Neutrophil elastase digests ferritin increasing the extracellular iron pool available for cellular uptake, it generates reactive oxygen species by increasing lung nonheme iron. Phenotypes of A1AT deficiency patinets and of rats with lungs injured with human neutrophil elastase, overview. The rats show iron-laden macrophages at day 28, but no sideromacrophages
malfunction
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inhibition of neutrophil elastase ameliorates mouse liver damage due to ischemia and reperfusion, overview
malfunction
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inhibition of neutrophil elastase is effective in reducing acute lung injury associated with systemic inflammatory response syndrome, SIRS
malfunction
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leukocyte elastase induces apoptosis in lung epithelial cells mediated by a PAR-1-triggered pathway involving activation of NF-kappaB and p53, and a PUMA- and Bax-dependent increase in mitochondrial permeability leading to activation of distal caspases, regulation mechanism, detailed overview
malfunction
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neutrophil elastase reduces the levels of coagulation factors and inhibits repair of following mechanical wounding of epithelial 16HBE 14o- cell layers. PAR-1 and PAR-2 peptide agonists stimulated the rate of repair and enhanced the formation of a fibrin provisional matrix to support the repair process, overview. But neutrophil elastase does not cleave the N-terminal domains and does not disarm either PAR-1 or PAR-2
malfunction
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pharmacologic inhibition of neutrophil elastase and genetic deletion significantly reduce infarct volume, blood-brain barrier disruption, vasogenic edema, and leukocyte-endothelial adherence after transient middle cerebral artery occlusion
malfunction
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release of neutrophil elastase is one of the harmful inflammatory reactions in acute cerebral ischemia
malfunction
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neutrophil elastase deficient mice have a significant survival advantage over wild type animals following exposure to anthrax lethal toxin
malfunction
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neutrophil elastase knockout mice do not form neutrophil extracellular traps in a pulmonary model of Klebsiella pneumoniae infection
malfunction
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neutrophil elastase knockout mice respond with increased lung permeability to infection with Streptococcus pneumoniae, resulting in severe respiratory distress and progressive mortality. Ablation of hematopoietic neutrophil elastase in bone marrow chimeras abolishes intra-alveolar neutrophil elastase immunoreactivity and leads to bacterial outgrowth in the lungs of mice
malfunction
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absence of neutrophil elastase increases host tolerance, rather than resistance, to lung infection with Burkholderia thailandensis and B. pseudomallei by minimizing host tissue damage
malfunction
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enzyme-deficient mice are protected from asbestos-induced lung fibrosis
malfunction
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mutations in the enzyme can either cause severe congenital neutropenia or cyclic neutropenia
malfunction
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mutations in the enzyme can either cause severe congenital neutropenia or cyclic neutropenia
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a putative role of NE in the toll-like receptor 4, TLR4, signal transduction pathway
physiological function
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leukocyte elastase is a serine protease stored in and secreted from neutrophils that plays a determinant role in the pathogenesis of several lung diseases
physiological function
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neutrophil elastase decreases the endothelial production of prostacyclin through the inhibition of endothelial nitric oxide synthase activation and thereby contributes to the development of ischemia/reperfusion-induced liver injury. Neutrophil elastase may play a critical role in the development of I/R-induced liver injury by decreasing the IGF-I production through the inhibition of sensory neuron stimulation, which may lead to an increase of neutrophil accumulation and hepatic apoptosis through activation of caspase-3 in rats, overview
physiological function
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neutrophil elastase may play a key role in the disintegration of the hoof basal membrane
physiological function
human neutrophil elastase plays an important role in cell signaling and represents an regulator of the inflammatory response
physiological function
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leukocyte elastase may contribute to the degradation of cross-linked fibrin in sepsis-induced disseminated intravascular coagulation
physiological function
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neutrophil elastase acts as a biased agonist for proteinase-activated receptor 2. Neutrophil elastase causes a tethered ligand-independent proteinase-activated receptor 2-mediated activation of MAPK that does not involve either receptor internalization or recruitment of beta-arrestin. The enzyme can disarm proteinase-activated receptor 2-dependent calcium responses to trypsin
physiological function
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neutrophil elastase directly damages the extracellular matrix and neutrophil elastase degradation of mouse BP180 generates neutrophil chemotactic peptides that amplify disease severity at the early stage of bullous pemphigoid. Local injection of neutrophil elastase induces neutrophil infiltration
physiological function
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neutrophil elastase plays a critical and nonredundant role in lung-protective immunity against Streptococcus pneumoniae in mice
physiological function
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neutrophil elastase plays a key role in mediating the deleterious effects of anthrax lethal toxin. There is a marked lethal toxin-induced intestinal ulceration and bleeding in wild type neutrophil elastase animals
physiological function
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neutrophil elastase promotes lung tumor growth in vivo and induces tumor cell proliferation which is dependent upon platelet derived growth factor receptor-phosphtidylinositol-3 kinase signaling
physiological function
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upon activation, neutrophil elastase escapes from azurophilic granules and translocates to the nucleus, where it partially degrades specific histones, promoting chromatin decondensation. Myeloperoxidase synergizes with neutrophil elastase in driving chromatin decondensation. Neutrophil elastase and myeloperoxidase regulate the formation of neutrophil extracellular traps
physiological function
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addition of neutrophil elastase to Trypanosoma cruzi-infected macrophages reduced the replication of the parasites
physiological function
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human leukocyte elastase is not a driving force for elastolysis, but may promote further breakdown of elastic fibers after the action of other enzymes such as cathepsin G
physiological function
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neutrophil elastase causes tissue damage and increases vascular leakage that decreases host tolerance to lung infection with Burkholderia thailandensis and B. pseudomallei
physiological function
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the enzyme induces fibroblast proliferation and promotes myofibroblast differentiation
physiological function
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the enzyme enhances the proliferation and decreases apoptosis of leukemia cells via activation of PI3K/Akt signaling. The enzyme significantly promotes the proliferation of NB-4 cells, inhibits cell apoptosis and apoptotic signaling, and leads the activation of Akt.