The activities of key soluble enzymes, (a, f) NTPDase1/CD39 (ADPase), (b, g) ecto-5′-nucleotidase/CD73, (c, h) alkaline phosphatase (AP), (d, i) adenylate kinase (AK), and (e, j) nucleotide pyrophosphatase phosphodiesterase (NPP) were determined in (a–e) the serum of healthy control individuals and PXE patients and in (f–j) Abcc6−/− and WT mice. Data represent the mean ± standard error of the mean of the indicated number of serum samples. #P < 0.05; ##P < 0.01. CTRL, control; IU, international unit; nM/mL/hr, nano molar/milliliter/hour; PXE, pseudoxanthoma elasticum.