Article
Modeling pulmonary alveolar microlithiasis by epithelial deletion of the Npt2b sodium phosphate cotransporter reveals putative biomarkers and strategies for treatment.
Science translational medicine - 11 Nov 2015
Saito Atsushi, Nikolaidis Nikolaos M, Amlal Hassane, Uehara Yasuaki, Gardner Jason C, LaSance Kathleen, Pitstick Lori B, Bridges James P, Wikenheiser-Brokamp Kathryn A, McGraw Dennis W, Woods Jason C, Sabbagh Yves, Schiavi Susan C, Altinişik Göksel, Jakopović Marko, Inoue Yoshikazu, McCormack Francis X
Abstract excerpt
Pulmonary alveolar microlithiasis (PAM) is a rare, autosomal recessive lung disorder associated with progressive accumulation of calcium phosphate microliths. Inactivating mutations in SLC34A2, which encodes the NPT2b sodium-dependent phosphate cotransporter, has been proposed as a cause of PAM. We show that epithelial deletion of Npt2b in mice results in a progressive pulmonary process characterized by diffuse...
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