TABLE 1

Main characteristics of elastase, proteinase 3, and cathepsin G

CharacteristicElastaseProteinase 3Cathepsin G
EC number3.4.21.373.4.21.763.4.21.20
Gene locus and gene structure19p13.3, 5 exons 4 introns19p13.3, 5 exons 4 introns14q11.2, 5 exons 4 introns
Endogenous activatorDipeptidyl peptidase IDipeptidyl peptidase IDipeptidyl peptidase I
    ProdipeptideSer-GluAla-GluGly-Glu
Characteristics of mature forms
    Residues218222235
    Molecular mass29–33 kDa29–32 kDa28.5 kDa
    pI∼10.5∼9.5∼12
    Number of glycosylation sites221
    Number of disulfide bridges443
Optimal pH for activity8.0–8.5∼8.0∼7.5
Substrate specificitySmall hydrophobic residue at P1 position: Val, Cys, Ala, Met, Ile, Leu, SerSmall hydrophobic residue at P1 position: Val, Cys, Ala, Met, Ser, LeuAromatic or positively charged residues at P1 position: Phe, Tyr, Lys, Arg
Best FRET substrateAbz-APEEIMRRQ-EDDnpAbz-VADnorVADYQ-EDDnpAbz-EPFWEDQ-EDDnp
Localization in neutrophilAzurophilic granulesAzurophilic granulesAzurophilic granules
Nuclear envelopeSpecific granulesNeutrophil cell surface after priming
Neutrophil cell surface after primingSecretory vesiclesNeutrophil extracellular traps
Neutrophil extracellular trapsOn quiescent and activated neutrophil cell surface
Neutrophil extracellular traps
SourceNeutrophilNeutrophilNeutrophil
MonocyteMonocyteMonocyte
BasophilMastocyte
Endogenous inhibitorsα2-Macroglobulinα2-Macroglobulinα2-Macroglobulin
α1-PI/MNEI/PI9α1-PI/MNEIACT/MNEI/α1-PI/PI6
SLPI/Elafin/pre-elafinElafin/pre-elafinSLPI
Biologicals functionsDegradation of ECM componentsDegradation of ECM componentsDegradation of ECM components
Bactericidal propertiesBactericidal propertiesBactericidal properties
Cleavage of inflammatory mediatorsCleavage of inflammatory mediatorsCleavage of inflammatory mediators
Cleavage of receptorsCleavage of receptorsCleavage of receptors
Cleavage of lung surfactantInduction of endothelial cell apoptosisPlatelet activation
Cytokine and chemokine inductionNegative feedback regulation of granulopoiesisInduction of airway submucosal gland secretion
Induction of airway submucosal gland secretion
Pathological roles in human diseasesInflammatory diseasesInflammatory diseasesInflammatory diseases
Papillon-Lefèvre syndromePapillon-Lefèvre syndromePapillon-Lefèvre syndrome
Hereditary neutropeniaWegener's granulomatosis