Newspaper article The Canadian Press

Scientists Grow Lung Cells from CF Patients in Lab with Aim of Testing Drugs

Newspaper article The Canadian Press

Scientists Grow Lung Cells from CF Patients in Lab with Aim of Testing Drugs

Article excerpt

Lung cells grown in lab to test CF drugs

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TORONTO - Researchers have produced lung cells in the lab using stem cells grown from the skin of patients with cystic fibrosis -- a tool they believe can be used to test drugs that might overcome the debilitating, life-shortening condition.

In a study published Sunday in Nature Biotechnology, researchers at the Hospital for Sick Children in Toronto describe how they produced what are known as induced pluripotent stem cells from skin taken from patients with CF.

Induced pluripotent stem cells, or iPS cells, are adult cells that have been genetically manipulated in the lab to act like embryonic stem cells. As such, they can give rise to heart, muscle, liver and virtually any other cell type in the body.

In this case, the researchers wanted to make lung cells because it is the lungs that are primarily affected by cystic fibrosis.

One in every 3,600 children born in Canada has CF. The disease, which results from genetic mutations, causes a thick, sticky mucus to clog the lungs and airways, leading to repeated respiratory infections and lung damage.

The mucus also severely affects digestion: CF patients must take large doses of digestive enzymes at each meal so their bodies can absorb nutrients.

"What we would like to be able to do is take cells from patients, turn them into these iPS, or pluripotent cells, and then be able to turn them into lung cells that we can use to test for drugs that might help to treat cystic fibrosis," said principal researcher Janet Rossant, chief of research at Sick Kids.

Because a patient's cells all carry the genetic mutations that give rise to cystic fibrosis -- including the lung cells grown in the lab -- researchers can test drugs that specifically target the individual.

One of those drugs is an experimental compound currently being tested in the lab to see if it can reverse the effects of a key mutation in cystic fibrosis, which involves a gene known as CFTR.

The CFTR gene was isolated at Sick Kids in 1989 by a team led by Lap-Chee Tsui.

"This study shows the major impact stem cell research can have on the field of individualized medicine," said Rossant. …

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