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elastase-2; neutrophil elastase; leukocyte elastase; medullasin; bone marrow serine protease ( ELA2, ELANE)
Function:
- modifies functions of natural killer cells, monocytes & granulocytes
- inhibits C5a-dependent neutrophil enzyme release & chemotaxis
- hydrolysis of proteins, including elastin
- preferential cleavage: Val-|-Xaa > Ala-|-Xaa
Structure:
- belongs to the peptidase S1 family, elastase subfamily
- contains 1 peptidase S1 domain
Expression: bone marrow cells
Pathology:
- defects in ELA2 are a cause of cyclic hematopoiesis
Genetics:
- proteinase 3, neutrophil elastase & azurocidin are within an approximately 50-kb cluster on chromosome 19p
Laboratory:
- leukocyte elastase in plasma
Related
ELA2 gene mutation
Specific
elastase-2B; pancreatic elastase IIB (ELA2B, CELA2B)
General
elastase
glycoprotein
secreted protein
Properties
SIZE: entity length = 267 aa
MW = 29 kD
COMPARTMENT: inner mitochondrial membrane
CELL: neutrophil
erythroblast
MOTIF: signal sequence {1-27}
S1 domain {30-247}
MOTIF: cysteine residue {C55}
MODIFICATION: cysteine residue {C71}
histidine residue {H70}
cysteine residue {C71}
MODIFICATION: cysteine residue {C55}
N-glycosylation site {N88}
aspartate residue {D117}
N-glycosylation site {N124}
cysteine residue {C151}
MODIFICATION: cysteine residue {C208}
N-glycosylation site {N173}
cysteine residue {C181}
MODIFICATION: cysteine residue {C187}
cysteine residue {C187}
MODIFICATION: cysteine residue {C181}
cysteine residue {C198}
MODIFICATION: cysteine residue {C223}
serine residue {S202}
cysteine residue {C208}
MODIFICATION: cysteine residue {C151}
cysteine residue {C223}
MODIFICATION: cysteine residue {C198}
Database Correlations
OMIM correlations
MORBIDMAP 130130
UniProt P08246
Pfam PF00089
Entrez Gene 1991
ENZYME 3.4.21.37
References
- OMIM :accession 130130
- UniProt :accession P08246
- ELA2base; ELA2 mutation db
http://bioinf.uta.fi/ELA2base/
- GeneReviews
https://www.genecards.org/cgi-bin/carddisp.pl?gene=ELA2
- Wikipedia; Note=elastase entry
http://en.wikipedia.org/wiki/elastase