Hedgehog-interacting protein orchestrates alveologenesis and protects against bronchopulmonary dysplasia and emphysema.

ABSTRACT: Most of the lung's gas-exchange surface forms during alveologenesis and its disruption causes bronchopulmonary dysplasia (BPD) in infants, characterized by alveolar simplification and myofibroblast accumulation. BPD also increases the risk of adult emphysema, marked by alveolar loss. Despite this connection, mechanisms linking these conditions and effective treatments are still lacking. We identify hedgehog-interacting protein (HHIP), associated with both BPD and emphysema, as a critical regulator of alveologenesis. During this process, Hhip-expressing cells expanded, accompanied by hedgehog (Hh) signaling inhibition and myofibroblast transition. Stromal-specific Hhip deletion led to hyperactivation of Hh-IGF1 signaling axis, causing persistent SMA⁺ myofibro

SUBMITTER: Ye D 

PROVIDER: S-EPMC12057671 | biostudies-literature | 2025 May

REPOSITORIES: biostudies-literature