Cystic Fibrosis Transmembrane conductane Regulator

Here is an image of the CFTR Channel at an open stage. In the image to the right, taken from an online source (http://old.schoolscience.co.uk/content/5/biology/mrc/3/page3.html), is a cross section of an epithelial cell membrane.

Bill's description of the CF gene for the CFTR image:

"The CF gene codes for the Cystic Fibrosis Transmembrane conductance Regulator or CFTR for short. The CFTR is found on smooth tissue throughout the body, most importantly in the airways and the pancreas. The CFTR is an ion channel that helps move water into the mucus in the lungs making it less viscous and easier to clear from the airways. While there are numerous mutations in the CF gene they all lead to a dysfunctional CFTR that is inable to pump ions across the membrane onto the surface of the lungs. This dysfunctional ion channel (CFTR) leads to mucus that is too thick for the cilia to move out of the lungsand the mucus builds up."