Cystic Fibrosis
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| What type of inheritance in CF? | Autosomal dominant
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| What to organs/ systems does CF affect? | Lungs + GIT
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| What is the result of the defective gene? | Production of unusually thick sticky mucus
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| What are the two main results of this think sticky mucus? | Clogs the lungs and clogs the pancreas
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| What is the result of the mucus clogging the lungs? | Life-threatening lung infections
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| What is the result of the mucus clogging the pancreas? | Stops enzymes being released that help break down and absorb food
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| What are the six symptoms? 1. Taste? 2. Lungs? (3) 3. Poor...but good...? 4. Frequent...? | 1. Salty tasting skin
2. Coughing, wheezing and frequent lung diseases
3. Poor growth but good apetite
4. Frequent greasy, bulky stools
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| Sweat glands (normal) - Fluid produced in secretory coil is rich in? | NaCl
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| Sweat glands (normal) - NaCl secretion is modified in the duct by? | NaCl reabsorption (without H2O)
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| Sweat glands (normal) - If NaCl is reabsorbed without H2O, the solution becomes more hypertonic/hypotonic/ same? | Hypotonic
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| Sweat glands (normal) - The duct lumen has a -ve/+ve/ same electrical charge when compared to outside the duct? | -ve
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| Sweat glands (normal) - So lumen is -vely charged, how do Na+ ions get reabsorbed? | Actively pumped out
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| Sweat glands (normal) - So lumen is -vely charged, how is cl- reabsorbed? | Passive movement through Cl- channels
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| Sweat glands (CF) - Therefore what is the pathophysiology in CF? | Defect Cl- channels (with low permeability) -> secretion remains -vely charged.
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| Pancreas duct cell (normal) - What is actively pumped out of the apical membrane into the lumen? | Cl-
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| Pancreas duct cell (normal)- Is Cl- being pumped into lumen active/ passive? | Active
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| Pancreas duct cell (normal) - HCO3- is needed in the stomach to do what? | Neutralize stomach acid
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| Secretory glands (normal)- Is HCO3- pumped into/ out of the lumen? | Into
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| Secretory glands (normal)- What is HCO3- exchanged for when pumped into the lumen? | Cl-
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| Pancreas duct cell (normal)- When being exchanged for HCO3-, does Cl- move up/down its concentration gradient? Why? | moves down its concentration gradient due to active pumping of Cl- into lumen
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| Pancreas duct cell (CF)- What does a deficient cAMP/Cl- pump lead to? (2) | Decreased Cl- to exchange for HCO3-, therefore less HCO3- in secretion (in lumen)
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| Pancreas duct cell (CF)- What does decreased HCO3- in secretion lead to? (2) -> (1) | Mucins are poorly solublized and less transportable -> stasis of mucus
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| What gene codes for the chloride channel? | CFTR gene
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| What is the Cl channel activated by? | Phosphorlyation
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| What is the genetic mutation present in CFTR gene that causes CF? (1) | Deletion of three bases
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| What does the deletion of 3 bases in the CTFR gene lead to? (3) | Misfolding of channels -> degredation of channels --> decreased concentration of Cl- channels in CF patients
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| How could gene therapy be used as a potential cure for CF? | Replace the faulty CFTR gene with a normal one
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| *For treatments see flow |
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| Lungs (normal) - What is the role of CFTR channels? | Secretion of Cl- (fluid)
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| Lungs (normal) - What is the role of ENaC channels? | Absorption of Cl- (fluid)
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| Lungs (normal) - What is the interaction between CFTR and ENaC? | Work in balance
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| Lungs (CF) - What does the down regulation of CFTR lead to? (Increased/ decreased -> ?) | Decreased secretion and increased absorption leading to volume depletion
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| Lungs (CF) - What does volume depletion lead to? | Decreased mucociliary clearance
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| Lungs (CF) - What is the treatment? | Block ENaC channels -> No absorption or secretion
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