Epithelial cell-adhesion protein cadherin 26 is dysregulated in congenital diaphragmatic hernia and congenital pulmonary

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ORIGINAL ARTICLE

Epithelial cell‑adhesion protein cadherin 26 is dysregulated in congenital diaphragmatic hernia and congenital pulmonary airway malformation Richard Wagner1,3 · Henry Li1 · Lojine Ayoub1 · Shana Kahnamoui1 · Daywin Patel1 · Camelia Stefanovici2 · Martin Lacher3 · Richard Keijzer1  Accepted: 20 October 2020 © Springer-Verlag GmbH Germany, part of Springer Nature 2020

Abstract Background  Congenital diaphragmatic hernia (CDH) and congenital pulmonary airway malformation (CPAM) are two inborn pathologies of the lung of unknown origin. Alterations of gene expression in airway epithelial cells are involved in the pathobiology of both diseases. We previously found decreased expression of the epithelial cell adhesion protein cadherin 26 (CDH26) in hypoplastic mice lungs. Here, our objective was to describe the expression and localization of CDH26 in hypoplastic CDH lungs and hyperproliferative CPAM tissues. Methods  After ethical approval, we used human lung tissues from CDH and CPAM cases and age-matched control samples from a previously established biobank. Furthermore, lungs from the nitrofen rat model of CDH were included in the study. We performed immunohistochemistry and western blot analysis with antibodies against CDH26 to examine protein localization and abundance. Statistical analysis was performed using Mann–Whitney U test with significance set at p