BiofilmsONLINE.com : News : Researchers Uncover a... -
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Published on: 6/26/2001
Last Visited: 7/9/2008
"We now have a clearer picture of why the protein doesn't function properly in the milder form of cystic fibrosis - segments of the protein are sticking together, interfering with the flow of chloride in and out of the cell," said Dr. Charles Deber, the study's principal investigator, a senior scientist in the HSC Research Institute and a professor of Biochemistry at the University of Toronto.Cystic fibrosis is not just one distinct phenotype, as researchers have found hundreds of different mutations in the CF gene and the corresponding protein.The milder form of cystic fibrosis, also known as pancreatic sufficient CF, occurs in 10-15 per cent of CF patients.In this form of the disease, which often manifests as lung disease and male infertility, CFTR is able to carry out partial function.In the more severe forms of cystic fibrosis, the protein never assembles properly and no chloride can be transported."This opens the door for new avenues of research in terms of rational drug design," added Dr. Deber."We will look for molecules that could possibly break up the abnormal bond that is causing the protein strands to stick together." CFTR is a membrane protein with a massive and complicated structure.Membrane proteins, which regulate the stable internal physiological conditions of the cell in a controlled way, allowing for such things as intracellular communication, are very difficult to study. "We had to develop and refine molecular biological techniques in order to accurately study this membrane protein.This research will add to the body of knowledge about membrane proteins, which are also implicated in other diseases such as cancer, diabetes, and hypertension," said Dr. Alex Therien, the study's lead author and a Canadian Institutes of Health Research (CIHR) postdoctoral fellow in Dr. Deber's lab.
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The other author on this paper is Fiona Grant, who was student in Dr. Deber's lab as part of the Samuel Lunenfeld Research Summer Student Program in the HSC Research Institute.