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Initiation of CFTR Modulator

Children's Hospital Medical Center, Cincinnati · FDA-approved active Small molecule

CFTR modulators restore function to the cystic fibrosis transmembrane conductance regulator (CFTR) protein, improving chloride ion transport across cell membranes.

CFTR modulators restore function to the cystic fibrosis transmembrane conductance regulator (CFTR) protein, improving chloride ion transport across cell membranes. Used for Cystic fibrosis (specific mutations dependent on modulator type).

At a glance

Generic nameInitiation of CFTR Modulator
Also known as129Xe
SponsorChildren's Hospital Medical Center, Cincinnati
Drug classCFTR modulator
TargetCFTR (cystic fibrosis transmembrane conductance regulator)
ModalitySmall molecule
Therapeutic areaPulmonary/Genetic
PhaseFDA-approved

Mechanism of action

CFTR modulators work by either correcting the trafficking and localization of defective CFTR protein to the cell surface (correctors) or enhancing the channel-opening activity of CFTR at the membrane (potentiators). This restores the ability of epithelial cells to properly regulate salt and water balance, reducing the thick mucus secretions characteristic of cystic fibrosis.

Approved indications

Common side effects

Key clinical trials

Primary sources

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SourceUsed for
ClinicalTrials.govTrial enrolment, design, endpoints, results

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