Ectopic calcification in pseudoxanthoma elasticum responds to inhibition of tissue-nonspecific alkaline phosphatase

@article{Ziegler2017EctopicCI,
  title={Ectopic calcification in pseudoxanthoma elasticum responds to inhibition of tissue-nonspecific alkaline phosphatase},
  author={Shira G Ziegler and Carlos R Ferreira and Elena Gallo MacFarlane and Ryan C Riddle and Ryan Edward Tomlinson and Emily Y. Chew and LisaW. Martin and Chen-Ting Ma and Eduard A. Sergienko and Anthony B Pinkerton and J. L. Mill{\'a}n and William A Gahl and Harry C Dietz},
  journal={Science Translational Medicine},
  year={2017},
  volume={9}
}
Biallelic mutations in ABCC6 cause pseudoxanthoma elasticum (PXE), a disease characterized by calcification in the skin, eyes, and blood vessels. The function of ATP-binding cassette C6 (ABCC6) and the pathogenesis of PXE remain unclear. We used mouse models and patient fibroblasts to demonstrate genetic interaction and shared biochemical and cellular mechanisms underlying ectopic calcification in PXE and related disorders caused by defined perturbations in extracellular adenosine 5… CONTINUE READING
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