The “cystic fibrosis transmembrane conductance regulator (CFTCR or CFTR) gene mutation results in the abnormal expression of cystic fibrosis transmembrane conductance regulator protein, which is a cyclic adenosine monophosphate (cAMP)-activated chloride channel present on the surface of many types of epithelial cells including those lining the airways, bile ducts, the pancreas, sweat ducts, and the vas deferens” (McCance et al., 2014, p. 1311). Abnormalities with the transportation of electrolytes across the cell membrane, affects the secretion of chloride and sodium in the sweat and abnormal thick secretions in the lungs, pancreas, and reproductive organs (Lawton & Schub, 2015).
McCance et al. (2014) also state the goblet cells and submucosal glands which are the mucus-secreting airway cells and increased in size and number and that an increased chloride excretion and sodium absorption bring about dehydration of the airway mucus that enhances the thick mucus that adheres to the epithelium making it hard to cough up the secretions and thusly encourages growth of bacteria. McCance et al. (2014) also report inflammation as evident by increased amount of IL-1 and Il-8 and state long-term damage is done to the respiratory system due to substantial amounts of neutrophils releasing oxidants like proteases which breaks down proteins like elastin and entice airway cells to produce IL-8 which attracts more neutrophils which means more inflammation and a vicious cycle. This protease also destroys IgG and components necessary for opsonization and phagocytosis and stimulates the mucus cells to make more mucus (McCance et al., 2014). Every individual has two CF genes called the cystic fibrosis transmembrane conductance regulator (CFTR) and that person must receive two copies of the CFTR mutated gene to have CF (CCF, n.d.).
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