CF_pathology_2008

CF_pathology_2008 - CF Pathology and Treatment How does a...

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Unformatted text preview: CF Pathology and Treatment How does a defect in a chloride channel affect the airway epithelium? What mutations cause CF? How does a mutation in the gene affect localization and function of the protein? If we can better understand fundamental cellular processes of ion homeostatis and protein processing, can we treat CF? Q: CF is a recessive autosomal disease. If the frequency of live births with CF is 1/2500 among Northern Europeans, what proportion of adults are carriers? A. 1/10 B. 1/25 C. 1/50 D. 1/100 E. 1/2500 Major Pathogenic Events in CF Lung Disease Absence/Dysfunction of CFTR Protein Mutations in CFTR Gene Altered Ion Transport Processes Altered Airway Surface Fluid Properties Infection Inflammation Loss of Respiratory Tissue "Vicious Cycle" Vicious cycle The CFTR protein forms a kinase- activated, cAMP-regulated chloride channel, expressed in many types of epithelial cells. Cl Water Na ~ Na K Cl Cl Na Chloride-Secreting Epithelium A)Increase the osmotic potential in the lumen and water would go into the lumen B)Increase the osmotic potential in the lumen and water would go out of the lumen C)Decrease the osmotic potential in the lumen and water would go into the lumen D)Decrease the osmotic potential in the lumen and water would go out of the lumen Q: Secretion of chloride and sodium ions to the lumen would: Water Na Cl Cl Water Na airway lumen serosal surface Normal Cells CF Cells In the absence of functional CFTR, overactive sodium-absorption leads to dehydration of the lumen The most devastating...
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CF_pathology_2008 - CF Pathology and Treatment How does a...

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