Flashcards
iNFECTION - LECTURE 3
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- 1Give some statistics regarding the epidemiology and brief aetiology of cystic fibrosis.1. 1 in 25 carrier rate and 10,000 sufferers.
2. Recessive, caused by mutations in CFTR.
3. Cystif Fibrosis Transmembrane Conductance Regulator (CFTR)
- Chloride channel on apical surface of endothelial cells.
- 1600 mutations identified, most common is deltaF508 - 2How does cystic fibrosis caused death?1. Chronic Lung Infection and Ultimately Respiratory Failure is the leading cause of morbidity and mortality.
2. Median age of survival is now approximately 40 years, although is heavily influenced by patient's microbiology status. - 3Explain how mucociliary clearance works?1. Airways are lined by ciliated epithelial cells and mucus.
2. Bacteria gets trapped in mucus
3. Beating action of cilia moves mucus and bacteria out of the airways. - 4How do antimicrobial peptides work?1. Epithelial cells produce cationic antimicrobial peptides.
2. Small (12-50 amino acids) peptides with potent antimicrobial activity.
3. Positively charged and hydrophobic
4. Interact and disrupt bacterial membrane.
5. Typically, activity is salt sensitive. - 5Describe function of CFTR. 1. ATP dependant anion channel.
- 6Explain the "low volume" hypothesis of CFTR.1. Inhibition of ENac by CFTR. When that inhibition is removed, you get dysregulated flow of large amounts of Na+ across the epithelial cells, accompanied by water.
Summary: Loss of CFTR results in dysregulated isotonic fluid absorption and thus dehydrated mucus. This inhibits mucociliary clearance.
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- N/A Infection Flashcards on iNFECTION - LECTURE 3, created by Danial Dadgostar on 01/01/2015.
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- Danial Dadgostar
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