Cores

Biofilm_P.Aeruginosa(En face view of P. aeruginosa biofilm (green) growing on organotypic culture of CF airway epithelial cells)

Airway Cells and Tissues (Core A)

This Core exploits the availability of primary human bronchial epithelia (HBE), cultured from tissue obtained at lung transplant. This essential resource has been expanded to include human nasal epithelia (HNE) and sinonasal epithelial cells (SNEC). These cultures enable studies of epithelial ion transport, ASL homeostasis, innate immunity and therapeutic response that explore less common CFTR genotypes and the impact of the sinuses in initiating infections.

Assays (Core B)

This Core’s primary goal is to provide functional assays of CFTR, ENaC and other CF relevant transporter pathways to Center investigators. The Core provides specialized assays of transport function and protein trafficking and of mucociliary transport, ciliary beat frequency, and properties of the ASL – height, viscosity and pH – to examine the impact of regulators, inflammatory cytokines and small molecules on the functional properties of differentiated airway tissues.

Infection and Immunity (Core C)

This Core develops models for host-pathogen interactions and biofilm growth using live-cell imaging of airway pathogens on the surface of CF respiratory epithelia. Immunologic assays monitor the pathogenic response of immune cells. Mouse models of acute and chronic infections are assessed histologically and by lung function and using immune pathway-targeted, epithelia-specific mouse lines. Next-generation sequence analysis explores how CFTR dysfunction leads to chronic infection and unsuitable immune responses, supported by bioinformatics and biostatistical support and integration with patient metadata.

Translational Studies (Core D)

This Core supports clinical studies using a team of clinical research coordinators and regulatory experts. Physiological measurements include human airway function, in vivo airway absorptive/mucociliary clearance scans and infant pulmonary function testing. A multi-level model of airway function is being developed with investigators in Chemical Engineering. The Core provides biostatistics and data analytics to support investigator-initiated CF studies, and supports clinical databases linked with patient samples in the biobank.