Cystic
fibrosis is an inherited genetic disease caused by a mutation in the CTFR protein.
The CTFR protein is a gene containing the instructions for creating a channel to
transport chloride ions in and out of cells. This basic exchange is how sweat and mucous are produced.
When the gene is mutated, it disrupts the epithelium of cells that make up sweat glands in the skin and lining the lungs, liver, pancreas, and digestive and reproductive systems. The epithelium can no longer regulate how chloride passes along the cell membrane, thus mucous becomes thicker. One of the problems with this is that it inhibits mucous' normal function - which is trapping germs and then getting cleared out of the lungs. Cystic fibrosis mucous is so thick that it traps germs but is unable to exit lungs. Germs stay put which can cause infections.
Cystic fibrosis is a genetic disease meaning it is passed on from parents to children through DNA. To have the disease, one
must have inherited the faulty, mutated CTFR
gene from both the mother AND the father.
