About this pathway
Introduction
Elexacaftor is a modulator of CFTR that is designed to be used in combination with ivacaftor and tezacaftor to counteract variants that impact the proper splicing, folding, and location of CFTR to the membrane (FDA Multi-Discipline review,Elexacaftor/Tezacaftor/Ivacaftor)[Articles:38249954, 38186649, 37983082]. Ivacaftor/tezacaftor/elexacaftor is FDA and EMA indicated for the treatment of cystic fibrosis in patients 6 years and older who have at least one F508del mutation.
Pharmacokinetics
Metabolism
Elexacaftor is metabolized in the liver by CYP3A4 and CYP3A5 and excreted primarily in bile (FDA Multi-Discipline review,Elexacaftor/Tezacaftor/Ivacaftor)[Articles:36036885, 37259485]. The major metabolite is elexacaftor metabolite m23, which has about one third activity towards CFTR compared to that of the parent drug (FDA Multi-Discipline review,Elexacaftor/Tezacaftor/Ivacaftor).
Transport
No transporters are reported where elexacaftor is a substrate however in vitro it is an inhibitor of SLCO1B1 and SLCO1B3 [Article:37259485] (FDA Multi-Discipline review,Elexacaftor/Tezacaftor/Ivacaftor).
Reactions & interactions (10)
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Biochemical Reaction
elexacaftor → elexacaftor metabolite m23
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Catalysis
CYP3A5 → Biochemical Reaction
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Catalysis
CYP3A4 → Biochemical Reaction
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Inhibition
elexacaftor → SLCO1B1
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Inhibition
elexacaftor metabolite m23 → SLCO1B1
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Inhibition
elexacaftor metabolite m23 → SLCO1B3
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Inhibition
elexacaftor → SLCO1B3
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Leads To
elexacaftor metabolite m23 → Ivacaftor pathway, pharmacokinetics/pharmacodynamics
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Leads To
Ivacaftor pathway, pharmacokinetics/pharmacodynamics → Cystic Fibrosis
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Leads To
elexacaftor → Ivacaftor pathway, pharmacokinetics/pharmacodynamics
Edit history (2)
- 2024-02-28 Create
- 2024-03-14 Update Added related pathways