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London scientists working with mice have discovered that by correcting an abnormal gene linked to cystic fibrosis the mouse has a normal two-year lifespan instead of dying at four or six weeks.
The hope is the research will eventually lead to a new treatment for the fatal genetic disorder that hits one in 3,600 Canadians.
"The important aspect of this will be the translation of this research from the mouse model back into the clinical setting into actual cystic fibrosis patients," said Dr. Richard Rozmahel of the Lawson Research Institute.
Rozmahel's research team looked at more than 30,000 genes to find ones different in a normal mouse from one in cystic fibrosis mice.
One gene with a different expression in cystic fibrosis mice was mCLCA3, a gene similar to one found in humans with cystic fibrosis.
In the CF mice, it was only being expressed at one-third the level of healthy mice.
When the Lawson scientists brought the gene up to normal expression, the mucous blockages in the lungs and intestines were reduced.
"They could survive normally," said Rozmahel.
The next step in their research will be to pinpoint exactly how the gene CLCA3 functions, he said.
"We have shown CLCA3 is important to cystic fibrosis but the function of CLCA3 is not fully understood," said Rozmahel.
"Is it acting on the properties of the mucous to allow the mucous to be cleared better, is it working on the hydration of the mucous so that mucous has more fluid in it so it would be cleared better, or is it acting to decrease secretion of the mucous by these cells?"
Individuals born with cystic fibrosis used to survive only a few years, but drug therapies and lung transplants have pushed the median life expectancy to 37.
The most obvious problem for individuals with the disease is the build of mucous in the lungs, which provides a breeding ground for bacteria.
"In cystic fibrosis the mucous is not able to be cleared because it is so thick and sticky," Rozmahel said.
"If we were able to promote the clearance of mucous we would be able to correct at least part of the problem."
London scientists working with mice have discovered that by correcting an abnormal gene linked to cystic fibrosis the mouse has a normal two-year lifespan instead of dying at four or six weeks.
The hope is the research will eventually lead to a new treatment for the fatal genetic disorder that hits one in 3,600 Canadians.
"The important aspect of this will be the translation of this research from the mouse model back into the clinical setting into actual cystic fibrosis patients," said Dr. Richard Rozmahel of the Lawson Research Institute.
Rozmahel's research team looked at more than 30,000 genes to find ones different in a normal mouse from one in cystic fibrosis mice.
One gene with a different expression in cystic fibrosis mice was mCLCA3, a gene similar to one found in humans with cystic fibrosis.
In the CF mice, it was only being expressed at one-third the level of healthy mice.
When the Lawson scientists brought the gene up to normal expression, the mucous blockages in the lungs and intestines were reduced.
"They could survive normally," said Rozmahel.
The next step in their research will be to pinpoint exactly how the gene CLCA3 functions, he said.
"We have shown CLCA3 is important to cystic fibrosis but the function of CLCA3 is not fully understood," said Rozmahel.
"Is it acting on the properties of the mucous to allow the mucous to be cleared better, is it working on the hydration of the mucous so that mucous has more fluid in it so it would be cleared better, or is it acting to decrease secretion of the mucous by these cells?"
Individuals born with cystic fibrosis used to survive only a few years, but drug therapies and lung transplants have pushed the median life expectancy to 37.
The most obvious problem for individuals with the disease is the build of mucous in the lungs, which provides a breeding ground for bacteria.
"In cystic fibrosis the mucous is not able to be cleared because it is so thick and sticky," Rozmahel said.
"If we were able to promote the clearance of mucous we would be able to correct at least part of the problem."