EC number | 3.4.21.37 | 3.4.21.76 | 3.4.21.20 |
Gene locus and gene structure | 19p13.3, 5 exons 4 introns | 19p13.3, 5 exons 4 introns | 14q11.2, 5 exons 4 introns |
Endogenous activator | Dipeptidyl peptidase I | Dipeptidyl peptidase I | Dipeptidyl peptidase I |
Prodipeptide | Ser-Glu | Ala-Glu | Gly-Glu |
Characteristics of mature forms | | | |
Residues | 218 | 222 | 235 |
Molecular mass | 29–33 kDa | 29–32 kDa | 28.5 kDa |
pI | ∼10.5 | ∼9.5 | ∼12 |
Number of glycosylation sites | 2 | 2 | 1 |
Number of disulfide bridges | 4 | 4 | 3 |
Optimal pH for activity | 8.0–8.5 | ∼8.0 | ∼7.5 |
Substrate specificity | Small hydrophobic residue at P1 position: Val, Cys, Ala, Met, Ile, Leu, Ser | Small hydrophobic residue at P1 position: Val, Cys, Ala, Met, Ser, Leu | Aromatic or positively charged residues at P1 position: Phe, Tyr, Lys, Arg |
Best FRET substrate | Abz-APEEIMRRQ-EDDnp | Abz-VADnorVADYQ-EDDnp | Abz-EPFWEDQ-EDDnp |
Localization in neutrophil | Azurophilic granules | Azurophilic granules | Azurophilic granules |
Nuclear envelope | Specific granules | Neutrophil cell surface after priming |
Neutrophil cell surface after priming | Secretory vesicles | Neutrophil extracellular traps |
Neutrophil extracellular traps | On quiescent and activated neutrophil cell surface | |
| Neutrophil extracellular traps | |
Source | Neutrophil | Neutrophil | Neutrophil |
Monocyte | Monocyte | Monocyte |
Basophil | Mastocyte |
Endogenous inhibitors | α2-Macroglobulin | α2-Macroglobulin | α2-Macroglobulin |
α1-PI/MNEI/PI9 | α1-PI/MNEI | ACT/MNEI/α1-PI/PI6 |
SLPI/Elafin/pre-elafin | Elafin/pre-elafin | SLPI |
Biologicals functions | Degradation of ECM components | Degradation of ECM components | Degradation of ECM components |
Bactericidal properties | Bactericidal properties | Bactericidal properties |
Cleavage of inflammatory mediators | Cleavage of inflammatory mediators | Cleavage of inflammatory mediators |
Cleavage of receptors | Cleavage of receptors | Cleavage of receptors |
Cleavage of lung surfactant | Induction of endothelial cell apoptosis | Platelet activation |
Cytokine and chemokine induction | Negative feedback regulation of granulopoiesis | Induction of airway submucosal gland secretion |
Induction of airway submucosal gland secretion | | |
Pathological roles in human diseases | Inflammatory diseases | Inflammatory diseases | Inflammatory diseases |
Papillon-Lefèvre syndrome | Papillon-Lefèvre syndrome | Papillon-Lefèvre syndrome |
Hereditary neutropenia | Wegener's granulomatosis | |