Actively Recruiting

Age: 12Years +
All Genders
NCT03161808

Rare CFTR Mutation Cell Collection Protocol (RARE)

Led by George Solomon · Updated on 2026-02-02

500

Participants Needed

1

Research Sites

582 weeks

Total Duration

On this page

Sponsors

G

George Solomon

Lead Sponsor

C

Cystic Fibrosis Foundation

Collaborating Sponsor

AI-Summary

What this Trial Is About

Over 1,900 mutations in the gene for the cystic fibrosis transmembrane conductance regulator (CFTR) protein are implicated in causing Cystic Fibrosis (CF). Potential therapies that directly target defective CFTR are being evaluated in important clinical trials, but most target the most common CFTR mutation F508del. Many patients with rare CF mutations are not able to participate in those studies. The RARE study is specifically designed for people with CF caused by rare mutations. Eligible rare mutations are listed below: • CF patients who are heterozygous for pre-mature stop codons as noted below: i. one allele must be a F508del ii. the other allele must be a pre-mature stop codon mutation • CF Patients with other genotypes that require Study PI permission: i. CF patients with two mutations that are not eligible for Trikafta ii. CF patients homozygous or heterozygous (other allele must be F508del) for rare mutations of special interest (e.g., 711+3A-\>G, 2789+5G-\>A, 3272-26A-\>G, 3849+10kbC-\>T). Other rare mutations will be considered on a case by case basis This is a multi-site, specimen collection study. Investigators will collect blood, intestinal cells and nasal cells from each participant. Cells from these specimens will be used to test future CFTR modulators to see if they might work for people with study eligible rare mutations. Having cells to test in the lab is an important first step in identifying potential new therapies for people with these mutations.

CONDITIONS

Official Title

Rare CFTR Mutation Cell Collection Protocol (RARE)

Who Can Participate

Age: 12Years +
All Genders

Eligibility Criteria

Eligible

You may qualify if you...

  • Male or female 12 years of age or older at time of consent
  • Documentation of a cystic fibrosis diagnosis with clinical features and one or more of the following: sweat chloride ≥ 60 mEq/L, two CFTR gene mutations, or abnormal nasal potential difference
  • Confirmed genotype focusing on certain rare mutations, initially homozygous pre-mature stop codons
  • Written informed consent (and assent when applicable) and ability to comply with study requirements
  • Willingness to travel to a regional study site for cell collection
Not Eligible

You will not qualify if you...

  • Medical conditions or abnormalities that may compromise data quality or pose significant risk during biopsy, including certain rectal/gastrointestinal diseases
  • Abnormal lab values such as platelets < 50 x 10^3/µL, hemoglobin < 10 gm/dL, hematocrit < 30%, WBC > 20 x 10^3/µL, neutropenia, lymphopenia, PT/INR > 1.5, or other bleeding disorders
  • Positive pregnancy test for females of childbearing potential at study visit
  • Breastfeeding if sedation is used
  • Use of immunosuppressive drugs like oral steroids > 20 mg/day for more than 14 days prior to biopsy
  • History of organ transplant
  • Use of oral anticoagulants within 7 days before biopsy
  • Inability or unwillingness to withhold oral anticoagulants for 7 days after biopsy

AI-Screening

AI-Powered Screening

Complete this quick 3-step screening to check your eligibility

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Trial Site Locations

Total: 1 location

1

University of Alabama at Birmingham

Birmingham, Alabama, United States, 35233

Actively Recruiting

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Research Team

H

Heather Y Hathorne, PhD

CONTACT

J

Justin A Anderson, MS

CONTACT

How is the study designed?

Study Type

OBSERVATIONAL

Masking

N/A

Allocation

N/A

Model

N/A

Primary Purpose

N/A

Number of Arms

0

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Rare CFTR Mutation Cell Collection Protocol (RARE) | DecenTrialz