Scientists at the University of Cambridge have successfully created a set of mini-lungs in a search for finding new treatments for those suffering from cystic fibrosis.
The mini-lungs, created using stem cells from patients suffering from the disease, function just like real lungs and could be used in testing drug therapies and treatments for the illness.
Cystic fibrosis is a life-threatening disease that mostly affects the lungs, creating layers of thick mucus in the airways, causing problems with breathing and increasing the chance of respiratory infection. Nearly 70,000 people worldwide suffer from the disease.
Cystic fibrosis is a genetic disease caused by a single gene mutation, although the specific gene involved often varies. People living with the disease can expect shorter life spans, although modern treatment has improved the odds for those who have the illness.
Researchers took skin cells from patients with the type of cystic fibrosis that's caused by a mutation in the CFTR gene. This is one of the most common types of the disease. Scientists then reprogrammed the cells and made them pluripotent, which means they can develop into several kinds of human cell. The research team grew these cells into embryonic lung cells and created distal airways, which is the part of the lung that's most often associated with cystic fibrosis.
"In a sense, what we've created are 'mini-lungs,'" says Dr. Nick Hannan, the study leader. "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."
To test their mini-lungs, scientists used a fluorescent dye sensitive to chloride. If the lungs worked, chloride would pass through the distal airways uninhibited in regular lungs, but not in those of patients with cystic fibrosis. In their experiments, the lungs worked (or didn't work) as expected. They also tested several drugs on the lungs created from patients with cystic fibrosis, showing that one improved cell functioning in those lungs.
"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," says Dr. Hannan. "This is far more practical, should provide more reliable data and is also more ethical than using large numbers of mice for such research."
