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myeloblastin; leukocyte proteinase 3; PR-3; PR3; azurophil granule protein 7; AGP7; Wegener autoantigen; P29; C-ANCA antigen; neutrophil proteinase 4; NP-4 (PRTN3, MBN)
Function:
- polymorphonuclear leukocyte serine protease
- degrades elastin, fibronectin, laminin, vitronectin, & collagen types I, III, & IV (in vitro)
- hydrolysis of proteins, including elastin, by preferential cleavage: -Ala-|-Xaa- > -Val-|-Xaa-
Structure:
- belongs to the peptidase S1 family, elastase subfamily
- contains 1 peptidase S1 domain
Expression:
- cytokines such as tumor necrosis factor may induce expression of myelobastin on the cell surface of neutrophils
Pathology:
- autoantigen of c-ANCA (cytoplasmic anti-neutrophil cytoplasmic antibody)
- in the presence of c-ANCA, neutrophils generate reactive oxygen species
- this would implicate c-ANCA in the pathology of Wegener's granulomatosis rather than simply as a marker of the disease
- causes emphysema when administered by tracheal insufflation to hamsters
Laboratory:
- proteinase 3 in serum
- proteinase-3 antibody in serum
Related
anti-neutrophil cytoplasmic antibody (ANCA)
General
antigen
serine protease trypsin family
Properties
SIZE: entity length = 256 aa
MW = 28 kD
MOTIF: signal sequence {1-25}
S1 domain {28-248}
MOTIF: cysteine residue {C56}
MODIFICATION: cysteine residue {C72}
histidine residue {H71}
cysteine residue {C72}
MODIFICATION: cysteine residue {C56}
aspartate residue {D118}
N-glycosylation site {N129}
cysteine residue {C152}
MODIFICATION: cysteine residue {C209}
N-glycosylation site {N174}
cysteine residue {C182}
MODIFICATION: cysteine residue {C188}
cysteine residue {C188}
MODIFICATION: cysteine residue {C182}
cysteine residue {C199}
MODIFICATION: cysteine residue {C224}
serine residue {S203}
cysteine residue {C209}
MODIFICATION: cysteine residue {C152}
cysteine residue {C224}
MODIFICATION: cysteine residue {C199}
Database Correlations
OMIM 177020
UniProt P24158
Pfam PF00089
Entrez Gene 5657
Kegg hsa:5657
ENZYME 3.4.21.76
References
- Medical Knowledge Self Assessment Program (MKSAP) 11, American
College of Physicians, Philadelphia 1998
- UniProt :accession P24158
- Wikipedia; Note: proteinase 3 entry
http://en.wikipedia.org/wiki/proteinase_3