Childhood interstitial lung diseases (chILD) are rare respiratory disorders that result in impaired gas exchange and increased mortality. This results in children requiring assisted breathing from a very early age and being at greater risk of severe lung infections. Our research aims to understand how genetic mutations cause cellular dysfunction resulting in chILD. We have uncovered the role of a new gene in the development of specialised lung cells and how this causes more severe viral infections in the lungs of these children.
reNEW research
At the Lung Disease lab at reNEW Melbourne, we generate iPSC-derived alveolar models from patients with chILD, paired with CRISPR gene-corrected isogenic controls. This design lets us confirm pathogenicity where clinical evidence alone is inconclusive. We characterise disease-relevant cellular phenotypes and use the same platform to screen approved and emerging drugs for efficacy. iPSCs are essential here since primary alveolar tissue from these rare, pediatric patients is otherwise inaccessible.
Image description
In this image, gene-edited iPSC-derived alveolar type II epithelial cells have been cultured in an air-liquid interface.
Credits
Jack Murphy, Research Assistant, Werder lab, reNEW Melbourne.
Katelyn Patatsos, Research Assistant, Werder lab, reNEW Melbourne.
