Access to CF Center Reagents & Capabilities

Resources currently available to the scientific community through the UAB CFRC:

A. Cystic Fibrosis Scientific Core Facilities   

NIH P30 Cell Model and Assay Core

    • Derives epithelial cell lines expressing wild type or mutant CFTR using lentiviral technologies (e.g. CF bronchial epithelial, HeLa, others)
    • Provides primary murine airway epithelial cells grown as monolayers,
    • Assists with patch clamp and single channel analysis of CFTR or other ion channels

Core Director: Kevin L. Kirk, Ph.D.
Phone: 205-934-3122

NIH P30 Animal Models Core

    • Generates murine models for studies of cystic fibrosis. These currently include:
      • Cftr-F508del muring line (B6.129S6 Cftrtm1Kth/J)
      • Two congenic Cftr knockout lines (Cftrtm1Unc knockout in C57BL/6 background and Cftrtm1Unc knockout in BALB/c background)
      • A mixed line expressing human CFTR-G542X under control of the intestine-specific FABP promoter on a Cftr knockout background [Cftrtm1Cam Tg(FABP hCFTR-G542X), Cftrtm1Cam Tg(FABP hCFTR-W1282X), and the corresponding Cftr knockout control (Cftrtm1Cam) lacking the FABP hCFTR-G542X transgene]
      • Cftrtm1G551D in 129 background onto the C57BL/6 background
      • Two congenic lines expressing wild type human CFTR under control of the intestine-specific Fatty Acid Binding Protein (FABP) promoter on a Cftr-/- background
      • Cftrtm1Unc Tg(FABPCFTR)1Jaw in C57BL/6J background
      • Cftrtm1Unc Tg(FABP CFTR)1Jaw in BALB/c background
    • Assists with mouse line generation by both oocyte micro-injection and embryonic stem cell gene targeting technologies
    • Furnishes electrophysiologic analysis of CFTR activation, including murine nasal potential difference assays and intestinal short circuit current measurements

Core Director: David M. Bedwell, Ph.D.
Phone: 205-934-9640

NIH P30 Clinical and Translational Core

    • Provides human primary nasal and bronchial airway epithelial cells. Available genotypes include:

      F508del/F508del F508del/unknown G551D/unknown G551D/F508del G551D/G551D
      G542X/unknown W1282X/unknown F508del/27895G>A F508del/R1162X F508del/R553X
      F508del/2184insA F508del/K710X F508del/R117H 5T/9T F508del/wt wt/wt

    • Provides expertise regarding the in vivo nasal potential difference measurement
    • Assists with design and implementation of CF clinical trials
    • Furnishes capabilities to study pulmonary tissue physiology utilizing optical coherence tomography (OCT)

Core Director: Steven M. Rowe, M.D.
Phone: 205-934-9640

CF Foundation - CFTR Expression Core

    • Assists with technologies necessary to efficiently express CFTR in experimental systems, including analysis of CFTR maturational efficiency, surface biotinylation, endoplasmic reticulum associated degradation, etc.
    • Maintains a large repository of plasmids for studying wild type and mutant CFTR including:

CFTR-GFP fusions, prokaryotic expression plasmids for individual CFTR domains (R-domain, nucleotide binding domains, portions of NBDs, carboxy and amino termini, cytoplasmic loops), eukaryotic expression plasmids (for synthesizing and purifying CFTR in mammalian cells), glycosylation mutants, epitope tags (HA, myc), and over 50 clinical mutations or small deletions that cause human CF

    • Assists with construction of vaccinia, adenovirus and other vectors for studies of CF related gene products

Core Director: Jeong S. Hong, Ph.D.
Phone: 205-975-8929

B. Specific Reagents from UAB available through the CFTR Folding Consortium:

  • 5A6.3 (CFTR monoclonal antibody)
  • 10B6.2 (CFTR monoclonal antibody)
  • HeLa cells expressing wt or F508-del CFTR from lentiviral promoter
  • CFTR R-Domain expression constructs

For provision of antibodies, purified CFTR domains, and other reagents relevant to studies of cystic fibrosis, click here to access the Cystic Fibrosis Folding Consortium website for ordering instructions.

C. Ordering Primary Human Airway Epithelial Cells

To order primary airway epithelial cells from CF and non-CF individuals, click here.