Cystic fibrosis COPY Flashcards
Ivacaftor
-CFTR potentiator: increase CFTR activity (targets class 3 mutations), binds to CFTR channel to increase probability of gate opening
- CFTR Modulator: Improve airway surface liquid layer
- Genetic testing is always required since they’re designed for specific mutations
Lumacaftor
- Acts a chaperone to improve CFTR folding
- CFTR corrector: Correct folding and trafficking (class 2 mutations)
- CFTR Modulator: Improve airway surface liquid layer
- Genetic testing is always required since they’re designed for specific mutations
Tezacaftor
- Alters CFTR protein to improve trafficking to cell surface
- CFTR corrector: correct folding & trafficking
- CFTR Modulator: Improve airway surface liquid layer
- Genetic testing is always required since they’re designed for specific mutations
Elexacaftor
- Alters CFTR protein to improve trafficking to cell surface
- CFTR corrector: correct folding & trafficking
- CFTR Modulator: Improve airway surface liquid layer
- Genetic testing is always required since they’re designed for specific mutations
Hypertonic Saline & Mannitol
Airway hydrators
Mucolytics
Inhaled n- acetylcysteine: cleaves disulfide linkages in mucus
Inhaled dornase alfa: DNAse endonuclease that cleaves long sticky DNA polymers in mucus to reduce sputum viscosity & elasticity
-CFTR potentiator: increase CFTR activity (targets class 3 mutations), binds to CFTR channel to increase probability of gate opening
- CFTR Modulator: Improve airway surface liquid layer
- Genetic testing is always required since they’re designed for specific mutations
Ivacaftor
- Acts a chaperone to improve CFTR folding
- CFTR corrector: Correct folding and trafficking (class 2 mutations)
- CFTR Modulator: Improve airway surface liquid layer
- Genetic testing is always required since they’re designed for specific mutations
Lumacaftor
- Alters CFTR protein to improve trafficking to cell surface
- CFTR corrector: correct folding & trafficking
- CFTR Modulator: Improve airway surface liquid layer
- Genetic testing is always required since they’re designed for specific mutations
Tezacaftor
- Alters CFTR protein to improve trafficking to cell surface
- CFTR corrector: correct folding & trafficking
- CFTR Modulator: Improve airway surface liquid layer
- Genetic testing is always required since they’re designed for specific mutations
Elexacaftor
Airway hydrators
Hypertonic Saline & Mannitol
Inhaled n- acetylcysteine: cleaves disulfide linkages in mucus
Inhaled dornase alfa: DNAse endonuclease that cleaves long sticky DNA polymers in mucus to reduce sputum viscosity & elasticity
Mucolytics