Cells, Год журнала: 2025, Номер 14(10), С. 687 - 687

Опубликована: Май 9, 2025

Congenital diaphragmatic hernia (CDH) is a complex disorder whereby improper formation of the diaphragm allows herniation internal organs into thoracic cavity, resulting in pulmonary hypoplasia among other complications. Although epithelial dysfunction central to CDH pathology, relatively little attention has been paid underlying mechanisms orchestrating malfunction. Proinflammatory signaling downstream impaired mechanotransduction due utero lung compression elucidated drive cell phenotypes. This illustrated by reduction nuclear YAP and upregulation NF-kB models. In this review, we draw from recent findings using emerging technologies examine discuss role as and, crucially, sufficient driver recognition limitations genetic knockout models recapitulate such heterogenic etiologically complicated disease, alternative established nitrofen rat model, air–liquid interface (ALI) cultures, organoids ex vivo explants. Throughout, acknowledge importance involving mechanical modeling order faithfully disease. Finally, explore novel therapeutic strategies stem regenerative therapies precision medicine defining endotypes guide treatments.

Язык: Английский