Transmembrane Regulator (CFTR) protein. The Cystic Fibrosis Transmembrane Regulator protein is an epithelial cell chloride channel found in epithelial cells. Cystic Fibrosis Transmembrane Regulator is found in epithelial cells that line the cavities of the human body that make secretions. Therefore, the epithelial cells line airways, bile ducts, pancreas, sweat ducts and vas deferens. At the cellular level defective chloride selection and excess sodium absorption occurs lead to dehydration. When dehydration occurs cilia mobility decreases, mucus adheres to the epithelium along with bacteria. In the airways release damaging oxidants and proteases that damage immunoglobulin which facilitates the phagocytosis of pathogens. The chain reaction includes chronic bacterial infection which leads to chronic neutrophilic inflammation and finally Bronchiectasis. Bronchiectasis is abnormal dilation of the bronchus is caused by recurrent infections of cystic fibrosis.
CYSTIC FIBROSIS GENETIC FACTORS AND PATHOPHYSIOLOGY 3 Cell membrane found in different parts of the body can be affected. In the skin cystic fibrosis can cause increased electrolyte level, dehydration and heat exhaustion. In the respiratory
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- Fall '17
- keisha lovence