# Core-003

> **NIH NIH P30** · CINCINNATI CHILDRENS HOSP MED CTR · 2021 · $231,301

## Abstract

The “Functional and Transcriptomic Atlas of CF Respiratory Epithelial Cells” seeks to establish a Core to
isolate and expand respiratory epithelial cells from both nasal and bronchial brushings from patients with
specific CFTR mutations. Cells will be cultured in bronchospheres and nasospheres. Fluid and electrolyte
secretion will be assessed in the cultures. Effects of CFTR mutations on cell-specific transcriptomes will be
studied at single cell level. Confocal microscopy and immunofluorescence will be used to carefully identify
the effects of CFTR mutations and “correctors” on cell differentiation, phenotypes, and functions. Detailed
cell-specific RNA databases will be produced for nasal and bronchial cell cultures using single cell RNA-seq.
Detailed molecular imaging, by confocal microscopy, will be used to create an Atlas of gene expression and
behavior related to CF. Effects of CFTR correctors on specific patient mutations will be explored in these in
vitro models.
Specific Aim 1 will isolate, culture, and evaluate respiratory epithelial cells for study of CF from bronchial
brushings. Specific Aim 2 will use NexGen single cell RNA-sequencing to create an Atlas of patient-specific
respiratory epithelial cell transcriptomics. These studies will identify the effects of CFTR mutations on gene
expression at the single cell level, identifying the effects of CFTR mutations and correctors on the behaviors
of individual cells of multiple cell types, including ciliated, serous, and goblet cells that will be present in these
cultures. The Core will create a molecular Atlas which will be cell-, patient-, and mutation-specific and will
provide an extensive data set for use by investigators active in the CF research community worldwide. Such
data will enable the ability to understand the pleotropic effects of CFTR, CFTR mutations, and CFTR
correctors in a patient-specific manner, as we move toward patient-specific therapies for cystic fibrosis.

## Key facts

- **NIH application ID:** 10249244
- **Project number:** 5P30DK117467-04
- **Recipient organization:** CINCINNATI CHILDRENS HOSP MED CTR
- **Principal Investigator:** John Paul Clancy
- **Activity code:** P30 (R01, R21, SBIR, etc.)
- **Funding institute:** NIH
- **Fiscal year:** 2021
- **Award amount:** $231,301
- **Award type:** 5
- **Project period:** 2018-08-01 → 2023-06-30

## Primary source

NIH RePORTER: https://reporter.nih.gov/project-details/10249244

## Citation

> US National Institutes of Health, RePORTER application 10249244, Core-003 (5P30DK117467-04). Retrieved via AI Analytics 2026-05-22 from https://api.ai-analytics.org/grant/nih/10249244. Licensed CC0.

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