Cystic Fibrosis Scientific Core Facilities   

NIH P30 Cell Model and Evaluation Core

Assists with patch clamp and single channel analysis of CFTR or other ion channels

Provides primary murine airway epithelial cells grown as monolayers

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

NIH P30 Animal Models Core

Cystic Fibrosis Murine Models
-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 adenovirus and other vectors for studies of CF related gene products< Conducts leading edge studies of gene suppression with siRNA and gene editing protocols using CRISPR/Cas9 or other methods

Core Director: Jeong S. Hong, Ph.D. and Eric Sorscher, M.D.
Phone: 205-975-8929

CF Foundation - CFTR Assay Core

-Provides Ussing chamber capabilities and expertise for testing vectoral anion transport and polarized airway epithelial monolayers
-Furnishes florescent dye-based methods for evaluating CFTR activity and airway surface liquid depth in CF cells and monolayers
-Performs immunolocalization for proteins relevant to CF pathogenesis, including CFTR
-Assists with leading edge transepithelial conductance measurements of CFTR activity

Core Director: Steve Rowe, M.D. and Cathy Fuller, Ph.D.
Phone: 205-934-9640

Ordering Primary Human Airway Epithelial Cells

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