Response to Article about Cystic Fibrosis Essay -- Biology

Cystic Fibrosis - Summary of Article It has been known for sort of some time that CFTR is a cAMP regulated chloride ion channel whose variant is intimately link up to cystic fibrosis (CF). However, the abnormality in the chloride ion channels is not the only biochemical peculiarity associated with the disease. Of particular interest to researchers is the finding that atomic number 11 ion (Na+) absorption in the cells of CF patients is both elevated and positively tie in to the cAMP concentration, whereas, in normal cells, cAMP concentration is inversely tie in to the rate of Na+ absorption. The malfunction of the Na+ sum becomes immediately apparent, for the primary election symptom of CF is a chronic bacterial infection of the lungs, overdue to the abnormally low viscosity of the fluid which bathes the cellular epithelium by pumping ions into the airway, rather than into the cell, a thermodynamically unfavorable concentration gradient of sodium ions is is established, in which the airway is hypotonic and osmosis is kinetically favored. In the case of CF, the local chloride pump is inoperative, and the sodium pumps are incredibly overactive, importing Na+ from the airway, and taking water out of the fluid inside the lungs in the carry out. Therefore, is is inappropriate to place the doctor responsibility for the low fluid viscosity directly upon the mutation of the CFTR protein. Studies stool demonstrated that the elevated Na+ absorption is not due to an overexpression of the Na+ transport protein, ENaC, in the membrane, nor cornerstone cAMP sensitivity be linked to a genetically mutated ENaC. The possibility that the altered CFTR indirectly led to the Na+ channel phenomena by altering the functionality of normal ENaC formed the basis of a hypothesis which ... ...The use of amiloride as a form of treatment is crucial because amiloride actually helps the body in correcting the chloride and sodium ion concentration, thereby rehydrating the cell s and thinning the mucus. This process differs from current treatments which loosen the mucus or dilute it so the patients can rids their lungs of the infectious substance more easily.Works Cited1.) Purves, WK, Orians GH, Heller HC. Life the lore of Biology. Sunderland Sinauer Associates, Inc. 1995. Chapter 15, Genetic Disease and Modern Medicine pages 334-352.2.) CF-FAQ Part 1. Ed. Ron Trueworthy. CF-WEB. Internet. 10 kinsfolk 1996.3.) S. H. Wright, interview by group members. Tucson, Arizona, 22 October 1996.4.) M. J. Stutts, C. M. Canessa, J. C. Olsen, M. Hamrick, J. A. Cohn, B. C. Rossier, R. C. Boucher, Science, v. 269, 11 August 1995, pages 847-850.