Stem cell models to decode chronic lung diseases

Project status: Active

Research area: Stem Cell Medicine > Lung Disease

A young girl has her lungs checked by a doctor

This study investigates chronic lung diseases using stem-cell models

This research leverages induced pluripotent stem cell (iPSC)-derived lung models from individuals with chronic lung diseases to explore disease mechanisms and immune responses during respiratory infections.

Purpose of our research

Respiratory infections are the leading cause of illness and death in individuals with chronic lung diseases, including alpha-1 antitrypsin deficiency (AATD) and cystic fibrosis (CF). Despite their significant health burden, there is a lack of targeted therapeutics to combat respiratory pathogens. Therefore, it is critical to expand our understanding of how chronic lung diseases interact with respiratory infections to inform the development of new treatments. Progress in this area has been hindered by limited access to lung samples from affected individuals.

Furthermore, variants of unknown significance (VUS) can be associated with respiratory illnesses in children but these pose challenges for diagnosis, prognosis, and therapeutic decision-making. Functional validation of these variants is essential to determine their pathogenicity and biological impact, enabling a clearer understanding of their role in disease mechanisms underlying childhood lung diseases.

Study details

This research aims to overcome this challenge by utilising iPSCs from donors with preexisting lung disease to investigate the mechanisms underlying disease development and immune responses to respiratory infections. We use iPSCs to create airway and alveolar epithelial cells, and innate immune cells (e.g., macrophages) to study lung development and respiratory infection responses.

This approach will offer valuable insights into the effects of emerging therapies on disease mechanisms and establish a predictive model for assessing novel therapeutics for people with chronic lung diseases.