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. 2019 Dec 17;20(24):6353. doi: 10.3390/ijms20246353

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© 2019 by the authors.

Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/).

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Systemic Pyrophosphate (PPi) synthesis. ABCC6 (expressed in hepatocytes and proximal tubular cells) and NPP1 (in arteries and capillaries) combined activity generates AMP in addition to PPi. AMP is rapidly converted into adenosine by CD73, which exerts a tonic inhibition on tissue non-specific alkaline enzyme (TNAP). TNAP degrades PPi to generate inorganic phosphate and promotes hydroxyapatite precipitation in ectopic tissues. ANK allows PPi externalization from cells, its role in ectopic calcifications is unclear.