RT Journal Article
T1 Plasma inorganic pyrophosphate and alkaline phosphatase in patients with pseudoxanthoma elasticum.
A1 Sánchez-Tévar, Ana María
A1 García-Fernández, María
A1 Murcia-Casas, Belén
A1 Rioja-Villodres, José
A1 Carrillo, Juan Luis
A1 Camacho, Marta
A1 Van Gils, Matthias
A1 Sánchez-Chaparro, Miguel Angel
A1 Vanakker, Olivier
A1 Valdivielso, Pedro
K1 CD73
K1 ENPP1
K1 Pseudoxanthoma elasticum (PXE)
K1 alkaline phosphatase (AP)
K1 calcification
K1 inorganic pyrophosphate (PPi)
K1 non-specific tissue alkaline phosphatase (TNAP)
AB Inorganic pyrophosphate (PPi) plays a major role inhibiting dystrophic calcification. The aim was to analyze levels of PPi in patients having pseudoxanthoma elasticum (PXE), and controls as well as the enzymes who regulate the PPi plasma concentration. We collected fasting blood samples from PXE patients and age- and sex-matched controls in ethylenediamine tetraacetic acid (EDTA) and citrate-theophylline-adenosine-dipyridamole (CTAD) containing tubes. We measured PPi, ENPP1 mass and activity, alkaline phosphatase (AP) and tissue non-specific alkaline phosphatase (TNAP), CD73 and Human Platelet Factor-4 (CXCL4). PPi in EDTA and CTAD samples were lower in PXE subjects than in controls (1.11±0.26 vs. 1.43±0.41 µM/L and 0.35±0.15 vs. 0.61±0.18 µM/L respectively, P High TNAP activity seems to contribute to low plasma levels of PPi in subjects with PXE, reinforcing the idea that pharmacological reduction of TNAP activity may help to reduce dystrophic calcification in PXE patients.
SN 2305-5839
YR 2019
FD 2019
LK https://hdl.handle.net/10668/27342
UL https://hdl.handle.net/10668/27342
LA en
DS RISalud
RD Apr 12, 2025