June 2017 - Vol 137 No 6

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Mutations in the gene encoding integrin a3 (ITGA3) cause interstitial lung disease with congenital nephrotic syndrome and junctional epidermolysis bullosa (ILNEB; MIM Number 614748). Pazzagli and coworkers studied primary and immortalized keratinocytes from ILNEB patients and determined that these cells had an activated phenotype. The laser confocal fluorescence microscopy image on the cover depicts a laminin 332-adherent HPV18 E6E7 immortalized ILNEB keratinocyte that displays several characteristic features. These include prominent focal adhesions and stress fibers. F-actin ¼ red; Vinculin ¼ green; and nucleus ¼ blue (DAPI). For details and other findings, see the article on page 1387.