Mini-lungs to help the study of cystic fibrosis

By using stem cells, scientists have created what they refer to as mini-lungs to aid cystic fibrosis studies.

Scientists at the University of Cambridge used stem cells derived from the skin of patients with cystic fibrosis – a lung disorder that triggers excessive mucus secretions – and successfully created the distal section of a lung.

“In a sense, what we’ve created are mini-lungs. While they only represent the distal part of lung tissue, they are grown from human cells and so can be more reliable than using traditional animal models, such as mice. We can use them to learn more about key aspects of serious diseases – in our case, cystic fibrosis,” said research leader, Dr Nick Hannan at the University of Cambridge.

In the study, published in the journal Stem Cells and Development, the team used stem cells from patients with the most common form of cystic fibrosis caused by a mutation in the CFTR gene. The scientists then reprogrammed the cells to a pluripotent state whereby the cells can develop into any type within the body.

Also, the team developed a method to test if the lung section is functioning properly by using a fluorescent dye sensitive to the presence of chloride. If functional, chloride would pass through the lung cells leading to a change in fluorescence. However, when they are malfunctioning cells from cystic fibrosis patients the fluorescence would not change.

The scientists believe this method could be used for disease modelling and drug testing, and plan to develop the technique for the study of other diseases.

Dr Hannan said: “We’re confident this process could be scaled up to enable us to screen tens of thousands of compounds and develop mini-lungs with other diseases such as lung cancer and idiopathic pulmonary fibrosis. This is far more practical, should provide more reliable data and is also more ethical than using large numbers of mice for such research”.

Paper: http://online.liebertpub.com/doi/10.1089/scd.2014.0512