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
NIH P30 Animal Models Core
Core Director: Kevin L. Kirk, Ph.D.
- Generates murine models for studies of cystic fibrosis. These currently include:
- Cftr-F508del murine 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 (The Core is currently backcrossing this strain which encodes an important disease-associated CFTR mutation) 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; and
- 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.
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, and 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.
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.
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.