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CFTR

Drugs affected by CFTR

Cystic Fibrosis Transmembrane Conductance Regulator

1 medication 1 brand product

About CFTR

CFTR is the chloride channel that's broken in cystic fibrosis. Modern CF therapies (ivacaftor, tezacaftor, elexacaftor, and their combinations) restore partial channel function — but only for patients whose specific CFTR variant produces a protein the drugs can act on. The FDA labels each modulator with the exact list of responsive variants.

Patients with at least one gating variant like G551D respond dramatically to ivacaftor. Patients homozygous for F508del benefit from the triple combination (Trikafta), but not from ivacaftor alone.

What we test for CFTR

Gene2Rx reports your CFTR genotype across 42 named alleles, built from 41 variants curated by PharmVar.

42
Named alleles
41
Variants tested
PharmVar
Source
GRCh38
Genome build
Unknown Function 1 ivacaftor responsive 38 ivacaftor non-responsive 2 ivacaftor irrelevant 1
What do these allele names mean?

CFTR alleles are named by the underlying DNA variant rather than a star number — for example c.61C>T describes a single base change at position 61 of the coding sequence.

You inherit one allele from each parent, and the pair determines whether your CFTR activity is normal, decreased, or absent.

PharmVar is the international registry that curates these names. Gene2Rx tests every variant needed to call each cataloged CFTR allele.

Medications with CFTR guidelines

Gene2Rx covers 1 medication with published pharmacogenetic guidance for CFTR, drawn from CPIC and FDA sources. Each drug links to its full pharmacogenetics page.

cystic fibrosis treatment

Brand products containing a CFTR-affected ingredient

These branded medications include at least one active ingredient whose metabolism or action involves CFTR. Each links to its full pharmacogenetic breakdown.

Find out your personal CFTR phenotype

This page lists drugs affected by CFTR. A Gene2Rx report tells you which metabolizer group you fall into, and what that means for every medication on this list.

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Informational only, not medical advice. The presence of a CFTR pharmacogenetic guideline does not mean every patient needs to change their dose. Never start, stop, or change a medication without talking to your prescribing clinician.

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