Research ArticleLung Disease

Modeling pulmonary alveolar microlithiasis by epithelial deletion of the Npt2b sodium phosphate cotransporter reveals putative biomarkers and strategies for treatment

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Science Translational Medicine  11 Nov 2015:
Vol. 7, Issue 313, pp. 313ra181
DOI: 10.1126/scitranslmed.aac8577

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Casting the first stone for lung disease

Pulmonary alveolar microlithiasis (PAM) is a rare lung disease characterized by spherical deposits of calcium phosphate. PAM is thought to be a genetic disorder, and mutations in the gene encoding the NPT2b sodium-dependent phosphate cotransporter have been implicated. Now, Saito et al. delete Npt2b in epithelial cells in mice and observe a disease that mimics human PAM. Whole-lung EDTA can reduce the burden of stones in the lungs, and a low-phosphate diet prevents stone formation. These data support a causative role of Npt2b in PAM and suggest strategies for treatment.

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