Neutrophil elastase

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Elastase 2, neutrophil
PDB rendering based on 1b0f.
Available structures: 1b0f, 1h1b, 1hne, 1ppf, 1ppg
Identifiers
Symbol(s) ELA2; HLE; HNE; NE; PMN-E
External IDs OMIM: 130130 MGI2679229 Homologene20455
RNA expression pattern

More reference expression data

Orthologs
Human Mouse
Entrez 1991 50701
Ensembl ENSG00000197561 ENSMUSG00000020125
Uniprot P08246 Q9Z284
Refseq NM_001972 (mRNA)
NP_001963 (protein)
NM_015779 (mRNA)
NP_056594 (protein)
Location Chr 19: 0.8 - 0.81 Mb Chr 10: 79.29 - 79.29 Mb
Pubmed search [1] [2]

Neutrophil elastase (or leukocyte elastase) is a type of enzyme that acts as a platelet activator. It consists of five exons. It is one of the two human forms of elastase.

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.

Neutrophil elastase is an important protease enzyme that when expressed aberrantly can cause emphysema or emphysematous changes. This involves breakdown of the lung structure and increased airspaces.


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.[1]


Contents

  • Dale DC, Liles WC, Garwicz D, Aprikyan AG (2002). "Clinical implications of mutations of neutrophil elastase in congenital and cyclic neutropenia.". J. Pediatr. Hematol. Oncol. 23 (4): 208-10. PMID 11846296. 
  • Horwitz M, Benson KF, Duan Z, et al. (2003). "Role of neutrophil elastase in bone marrow failure syndromes: molecular genetic revival of the chalone hypothesis.". Curr. Opin. Hematol. 10 (1): 49-54. PMID 12483111. 
  • Ancliff PJ, Gale RE, Linch DC (2003). "Neutrophil elastase mutations in congenital neutropenia.". Hematology 8 (3): 165-71. doi:10.1080/1024533031000107497. PMID 12745650. 
  • Horwitz M, Benson KF, Duan Z, et al. (2004). "Hereditary neutropenia: dogs explain human neutrophil elastase mutations.". Trends in molecular medicine 10 (4): 163-70. doi:10.1016/j.molmed.2004.02.002. PMID 15059607. 
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