Cystic Fibrosis Definition

Cystic Fibrosis Definition

 The CFTR protein helps to produce mucus. Mucus is a complex mixture of salts, water, sugars, and proteins that cleanses, lubricates, and protects many passageways in the body, including those in the lungs and pancreas. The role of the CFTR protein is to allow chloride ions to exit the mucus-producing cells. When the chloride ions leave these cells, water follows, thinning the mucus. In this way, the CFTR protein helps to keep mucus from becoming thick and sluggish, thus allowing the mucus to be moved steadily along the passageways to aid in cleansing.
In CF, the defective CFTR protein does not allow chloride ions out of mucus-producing cells. With less chloride leaving the cells, less water leaves, and mucus becomes thick and sticky. The mucus can no longer move freely through the passageways, and they become clogged. In the pancreas, clogged passageways prevent secretion of digestive enzymes into the intestine, causing serious impairment of digestion-especially the digestion of fat-which can lead to malnutrition. Mucus in the lungs may plug the airways, preventing good air exchange and, ultimately, leading to emphysema. The mucus is also a rich source of nutrients for bacteria, leading to frequent infections.

Cystic Fibrosis Definition

Cystic Fibrosis Definition

Cystic Fibrosis Definition

Cystic Fibrosis Definition

Cystic Fibrosis Definition

Cystic Fibrosis Definition

Cystic Fibrosis Definition

Cystic Fibrosis Definition

Cystic Fibrosis Definition