Fig. 1. ENPP1 deficiency leads to decreased levels of PP_i and elevated levels of ATP in the plasma of GACI individuals and ttw/ttw mice.

Plasma levels of PP_i (a, b) and ATP (c, d) were determined from healthy controls (n = 10) and GACI patients (n = 4) (a, c) and from 7-week-old WT (n = 6) and ttw/ttw mice (n = 5) (b, d). Plasma levels of ATP from healthy controls (c) correspond to normal human EDTA plasma concentrations from previous publications²⁷. Values are presented as the mean ± SD. ^**p < 0.005, ^***p < 0.001 (Student’s t test, unpaired two-sample testing for means)