Abstract
In brief: Generalized arterial calcification of infancy (GACI) is a severe, rare disease characterized by excessive calcification of large and medium sized arteries caused by homozygous loss-of-function mutations in ENPP1. Here, in Enpp1 deficient mice, treatment with recombinant human ENPP1 protein was more effective than bisphosphonates to resolve not only the vascular calcification, but also the hypertension that eventually leads to cardiac failure in GACI patients.
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