Assessment of ciliary phenotype in primary ciliary dyskinesia by micro-optical coherence tomography

Solomon, George M., Francis, Richard, Chu, Kengyeh K., Birket, Susan E., Gabriel, George, Trombley, John E., Lemke, Kristi L., Klena, Nikolai, Turner, Brett, Tearney, Guillermo J., Lo, Cecilia W., and Rowe, Steven M. (2017) Assessment of ciliary phenotype in primary ciliary dyskinesia by micro-optical coherence tomography. JCI Insight, 2 (5). e91702.

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Abstract

Ciliary motion defects cause defective mucociliary transport (MCT) in primary ciliary dyskinesia (PCD). Current diagnostic tests do not assess how MCT is affected by perturbation of ciliary motion. In this study, we sought to use micro-optical coherence tomography (μOCT) to delineate the mechanistic basis of cilia motion defects of PCD genes by functional categorization of cilia motion. Tracheae from three PCD mouse models were analyzed using μOCT to characterize ciliary motion and measure MCT. We developed multiple measures of ciliary activity, integrated these measures, and quantified dyskinesia by the angular range of the cilia effective stroke (ARC). Ccdc39-/- mice, with a known severe PCD mutation of ciliary axonemal organization, had absent motile ciliary regions, resulting in abrogated MCT. In contrast, Dnah5-/- mice, with a missense mutation of the outer dynein arms, had reduced ciliary beat frequency (CBF) but preserved motile area and ciliary stroke, maintaining some MCT. Wdr69-/- PCD mice exhibited normal motile area and CBF and partially delayed MCT due to abnormalities of ciliary ARC. Visualization of ciliary motion using μOCT provides quantitative assessment of ciliary motion and MCT. Comprehensive ciliary motion investigation in situ classifies ciliary motion defects and quantifies their contribution to delayed mucociliary clearance.

Item ID: 61122
Item Type: Article (Research - C1)
ISSN: 2379-3708
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Funders: National Institutes of Health (NIH), Cystic Fibrosis Foundation (CFF), Mucociliary Clearance Consortium
Projects and Grants: NIH P30 DK072482, NIH HL1116213, NIH HL098180, NIH GM104412, CFF CLANCY09Y0, CFF SORSCH15RO
Date Deposited: 04 Dec 2019 01:59
FoR Codes: 11 MEDICAL AND HEALTH SCIENCES > 1103 Clinical Sciences > 110316 Pathology (excl Oral Pathology) @ 20%
06 BIOLOGICAL SCIENCES > 0606 Physiology > 060602 Animal Physiology - Cell @ 20%
08 INFORMATION AND COMPUTING SCIENCES > 0801 Artificial Intelligence and Image Processing > 080106 Image Processing @ 60%
SEO Codes: 92 HEALTH > 9201 Clinical Health (Organs, Diseases and Abnormal Conditions) > 920115 Respiratory System and Diseases (incl. Asthma) @ 100%
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