Detection of CFTR function and modulation in primary human nasal cell spheroids.

ABSTRACT:

Background

Expansion of CFTR modulators to patients with rare/undescribed mutations will be facilitated by patient-derived models quantifying CFTR function and restoration. We aimed to generate a personalized model system of CFTR function and modulation using non-surgically obtained nasal epithelial cells (NECs).

Methods

NECs obtained by curettage from healthy volunteers and CF patients were expanded and grown in 3-dimensional culture as spheroids, characterized, and stimulated with cAMP-inducing agents to activate CFTR. Spheroid swelling was quantified as a proxy for CFTR function.

Results

NEC spheroids recapitulated characteristics of pseudostratified respiratory epithelia. When stimulated with forskolin/IBMX, spheroids swelled in the presence of functional CFTR, an

SUBMITTER: Brewington JJ 

PROVIDER: S-EPMC5868354 | biostudies-literature | 2018 Jan

REPOSITORIES: biostudies-literature