Bisphosphonates: from softening water to treating PXE

Stephanie N Moore, S Bobo Tanner, Jonathan G Schoenecker, Stephanie N Moore, S Bobo Tanner, Jonathan G Schoenecker

No abstract available

Keywords: ABCC6; Bisphosphonate; ENPP1; GACI; PXE; anti-mineralization; calcification; pyrophosphate.

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Figure 1.

At physiologic conditions calcium and phosphate are at saturating concentrations which should cause soft tissue mineralization. One mechanism of preventing aberrant mineralization is the 'pyrophosphate pump' which provides systemic pyrophosphate that inhibits calcium phosphate aggregation. Diseases such as PXE (ABCC6 mutation) are thought to cause insufficient circulating pyrophosphate leading to soft tissue mineralization. Li. et al. demonstrate that soft tissue mineralization can be prevented by supplementing pyrophosphate with non-hydrolyzable bisphosphonates.