ELANE

Elastases form a subfamily of serine proteases that hydrolyze many proteins in addition to elastin. Humans have six elastase genes which encode structurally similar proteins. The encoded preproprotein is proteolytically processed to generate the active protease. Following activation, this protease hydrolyzes proteins within specialized neutrophil lysosomes, called azurophil granules, as well as proteins of the extracellular matrix. The enzyme may play a role in degenerative and inflammatory diseases through proteolysis of collagen-IV and elastin. This protein also 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 present in a gene cluster on chromosome 19.

Elastase, Neutrophil Expressed

Modifies the functions of natural killer cells, monocytes and granulocytes. Inhibits C5a-dependent neutrophil enzyme release and chemotaxis (PubMed:15140022).

Capable of killing E.coli but not S.aureus in vitro; digests outer membrane protein A (ompA) in E.coli and K.pneumoniae (PubMed:10947984).

Acute inflammatory response to antigenic stimulus Source: GO_Central
Biosynthetic process of antibacterial peptides active against Gram-negative bacteria Source: UniProtKB
Cellular calcium ion homeostasis Source: UniProtKB
Defense response to bacterium Source: UniProtKB
Extracellular matrix disassembly Source: Reactome
Leukocyte migration involved in inflammatory response Source: Ensembl
Negative regulation of chemokine production Source: UniProtKB
Negative regulation of chemotaxis Source: UniProtKB
Negative regulation of inflammatory response Source: UniProtKB
Negative regulation of interleukin-8 production Source: UniProtKB
Negative regulation of transcription by RNA polymerase II Source: UniProtKB
Neutrophil-mediated killing of fungus Source: Ensembl
Neutrophil-mediated killing of gram-negative bacterium Source: UniProtKB
Phagocytosis Source: GO_Central
Positive regulation of immune response Source: Ensembl
Positive regulation of interleukin-8 production Source: UniProtKB
Positive regulation of leukocyte tethering or rolling Source: Ensembl
Positive regulation of MAP kinase activity Source: UniProtKB
Positive regulation of smooth muscle cell proliferation Source: UniProtKB
Protein catabolic process Source: UniProtKB
Proteolysis Source: UniProtKB
Pyroptosis Source: Reactome
Response to lipopolysaccharide Source: Ensembl
Response to UV Source: UniProtKB
Response to yeast Source: Ensembl

Phagosome. Localized in phagolysosomes following ingestion of E.coli by neutrophils.

Cyclic haematopoiesis (CH):
Autosomal dominant disease in which blood-cell production from the bone marrow oscillates with 21-day periodicity. Circulating neutrophils vary between almost normal numbers and zero. During intervals of neutropenia, affected individuals are at risk for opportunistic infection. Monocytes, platelets, lymphocytes and reticulocytes also cycle with the same frequency.
Neutropenia, severe congenital 1, autosomal dominant (SCN1):
A disorder of hematopoiesis characterized by maturation arrest of granulopoiesis at the level of promyelocytes with peripheral blood absolute neutrophil counts below 0.5 x 10(9)/l and early onset of severe bacterial infections.