# Functional Categorization of Ciliary Motion in PCD

> **NIH NIH K08** · UNIVERSITY OF ALABAMA AT BIRMINGHAM · 2021 · $159,882

## Abstract

PI: Solomon, George M.
 Primary Ciliary Dyskinesia (PCD) is a genetically heterogeneous disorder of motile cilia that results in
progressive lung disease due to abrogated mucociliary clearance. While new understandings of the genetics
have been helpful for clinical diagnostics, functional testing of cilia are needed to understand and predict
phenotypic variation and response to therapy. Recent work in our laboratory has identified a link between
ciliary genetics and novel ciliary phenotypes using one-micron optical coherence tomography (µOCT). We
have phenotyped the ciliary beating pattern of known and novel murine PCD models. We have extended this
work to humans through the use of human nasal epithelial cells. Using these technologies, we will
characterize and quantify functional ciliopathies My overall hypothesis is that PCD mutations can be
functionally analyzed to diagnose PCD, understand genotype-phenotype correlation on a mechanistic level,
and predict clinical response to ciliary agonists. The goals of this proposal are 1) to advance μOCT and other
functional imaging analysis to diagnose and categorize the functional consequences of PCD-associated gene
defects on Mucociliary transport in primary human cells and 2) determine whether pharmacological modulation
of ciliary function can augment ciliary motility and mucus clearance in mutants with motile cilia expression
through the following specific aims:
Specific Aim 1: Determine the diagnostic accuracy of µOCT-based functional analysis of primary
human airway cells.
Specific Aim 2: Determine the relationship between genotype and functional ciliary phenotype in
PCD. Specific Aim 3: Determine whether pharmacologic modulation of CBF can rescue MCT in PCD
with motile cilia expression.
 This project explores new concepts in the pathobiology of primary ciliary dyskinesia through the
innovative use of in vitro imaging (µOCT) measures of mucociliary transport and ciliary motion to diagnose and
precisely phenotype clinical PCD and the effect of genetics on ciliary function and MCT in human tissues. In
so doing, we will establish a protocol for testing of treatments to augment MCT and ciliary function. The
accompanying career development plan and the research aims as outlined above are of equal importance in
the development of this project. Combined with a strong mentoring committee, additional training in genetics
and cardiopulmonary physiology, study design, methodology, and statistical analysis, Dr. Solomon will have all
the tools to achieve his career goal as an independent physician-scientist. The opportunities created by this
career development award will result in the creation of a physician-scientist with the skills necessary to
accurately and ethically answer important scientific questions about potential therapies for PCD phenotypes,
successfully obtain future independent funding, and make important differences in the lives of PCD patients

## Key facts

- **NIH application ID:** 10078624
- **Project number:** 5K08HL138153-03
- **Recipient organization:** UNIVERSITY OF ALABAMA AT BIRMINGHAM
- **Principal Investigator:** George Martin Solomon
- **Activity code:** K08 (R01, R21, SBIR, etc.)
- **Funding institute:** NIH
- **Fiscal year:** 2021
- **Award amount:** $159,882
- **Award type:** 5
- **Project period:** 2019-01-07 → 2023-12-31

## Primary source

NIH RePORTER: https://reporter.nih.gov/project-details/10078624

## Citation

> US National Institutes of Health, RePORTER application 10078624, Functional Categorization of Ciliary Motion in PCD (5K08HL138153-03). Retrieved via AI Analytics 2026-05-21 from https://api.ai-analytics.org/grant/nih/10078624. Licensed CC0.

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