A Step Forward in Cystic Fibrosis Treatment

by Rajashri on Aug 1 2008 3:12 PM

A scientist from the Lawson Health Research Institute, affiliated with The University of Western Ontario, claims to have corrected the defect responsible for overproduction of intestinal mucous in cystic fibrosis.

Cystic fibrosis (also known as CF, mucoviscoidosis, or mucoviscidosis) is a hereditary disease that affects the exocrine (mucus) glands of the lungs, liver, pancreas, and intestines, causing progressive disability due to multisystem failure.

The research team led by Dr. Richard Rozmahel is investigating the potential of a gene found in mice, mCLCA3, which is similar to one in humans that exhibits abnormal levels in CF.

The mCLCA3 gene is expressed by cells that produce and secrete mucous.

They found that mCLCA3 plays an important role in the property of mucous, thereby allowing it to be cleared rather than result in the blockages that cause CF.

Correcting the abnormal levels of mCLCA3 in CF mice the researcher were able to overcome the mucous lesions. The correction also increased the survival rate in mice.

"It's my hope to understand what is causing the exaggerated mucous production and secretion in CF patients," said Dr. Rozmahel.

"From there, we can figure out ways to correct it," he added.