Table 1—

Cathepsin H concentrations, activities and inhibitor(cystatin C) concentrations, and ratios expressed on a molar basis

ControlPAP juvenilePAP adult
Cathepsin H
 Total nM203 (153–254)569 (452–686)500 (426–574)
 Activity nM7 (2–12)243 (88–399)467 (172–762)
Cystatin C
 Total nM1.0 (0.7–1.3)5.2 (3.2–7.2)5.6 (3.2–8.0)
Active cathepsin H %3 (1–5)46 (19–73)85 (40–129)
Ratios
 CatH–CatH activity85 (24–146)7.5 (1.6–13.3)15.2 (-13–43.3)
 CatH activity–CysC10.4 (1.3–19.6)41.6 (24.5–58.7)77.5 (48.2–107)
 CatH–CysC237 (163–310)276 (132–420)116 (70–163)
  • Data are presented as mean (95% confidence interval). PAP: pulmonary alveolar proteinosis; CatH: cathepsin H; CysC: cystatin C.