Person:
Leung, Hui

Profile Picture

Email Address

AA Acceptance Date

Birth Date

Research Projects

Organizational Units

Job Title

Last Name

Leung

First Name

Hui

Name

Leung, Hui

Filters

2016 - 20202

Settings

Author's Publications: search.filters.isAuthorOfPublication.b7d08482-58b9-4152-87c6-b3021e6da9d6

Search Results

Now showing 1 - 2 of 2
  • No Thumbnail Available
    Publication
    A human ciliopathy reveals essential functions for NEK10 in airway mucociliary clearance
    (Springer Science and Business Media LLC, 2020-01-20) Chivukula, Raghu R.; Montoro, Daniel T.; Leung, Hui; Yang, Jason; Shamseldin, Hanan E.; Taylor, Martin S.; Dougherty, Gerard W.; Zariwala, Maimoona A.; Carson, Johnny; Daniels, M. Leigh Anne; Sears, Patrick R.; Black, Katharine E.; Hariri, Lida P.; Almogarri, Ibrahim; Frenkel, Evgeni M.; Vinarsky, Vladimir; Omran, Heymut; Knowles, Michael R.; Tearney, Guillermo J.; Alkuraya, Fowzan S.; Sabatini, David M.
    Mucociliary clearance, the physiological process by which mammalian conducting airways expel pathogens and unwanted surface materials from the respiratory tract, depends on the coordinated function of multiple specialized cell types including basal stem cells, mucus-secreting goblet cells, motile ciliated cells, CFTR-rich ionocytes, and immune cells. Bronchiectasis, a syndrome of pathological airway dilation associated with impaired mucociliary clearance, may occur sporadically or with Mendelian inheritance, such as in cystic fibrosis (CF), primary ciliary dyskinesia (PCD), and select immunodeficiencies. Prior studies have identified mutations affecting ciliary structure and nucleation in PCD4, but the regulation of mucociliary transport remains incompletely understood and therapeutic targets for modulating it are lacking. Herein we identify a bronchiectasis syndrome caused by inactivating mutations in NEK10, a protein kinase with previously unknown in vivo function in mammals. Genetically modified primary human airway cultures establish NEK10 as a ciliated-cell specific kinase whose activity regulates the motile ciliary proteome to promote ciliary length and mucociliary transport, but which is dispensable for normal ciliary number, radial structure, and beat frequency. Together, these data identify a novel and likely targetable signaling axis which controls motile ciliary function in humans and has potential implications for other respiratory disorders characterized by impaired mucociliary clearance.
  • Thumbnail Image
    Publication
    Micro-optical coherence tomography of the mammalian cochlea
    (Nature Publishing Group, 2016) Iyer, Janani; Batts, Shelley A.; Chu, Kengyeh K.; Sahin, Mehmet I.; Leung, Hui; Tearney, Guillermo; Stankovic, Konstantina
    The mammalian cochlea has historically resisted attempts at high-resolution, non-invasive imaging due to its small size, complex three-dimensional structure, and embedded location within the temporal bone. As a result, little is known about the relationship between an individual’s cochlear pathology and hearing function, and otologists must rely on physiological testing and imaging methods that offer limited resolution to obtain information about the inner ear prior to performing surgery. Micro-optical coherence tomography (μOCT) is a non-invasive, low-coherence interferometric imaging technique capable of resolving cellular-level anatomic structures. To determine whether μOCT is capable of resolving mammalian intracochlear anatomy, fixed guinea pig inner ears were imaged as whole temporal bones with cochlea in situ. Anatomical structures such as the tunnel of Corti, space of Nuel, modiolus, scalae, and cell groupings were visualized, in addition to individual cell types such as neuronal fibers, hair cells, and supporting cells. Visualization of these structures, via volumetrically-reconstructed image stacks and endoscopic perspective videos, represents an improvement over previous efforts using conventional OCT. These are the first μOCT images of mammalian cochlear anatomy, and they demonstrate μOCT’s potential utility as an imaging tool in otology research.