PUBLICATION

Novel roles for the radial spoke head protein 9 in neural and neurosensory cilia

Authors
Sedykh, I., TeSlaa, J.J., Tatarsky, R.L., Keller, A.N., Toops, K.A., Lakkaraju, A., Nyholm, M.K., Wolman, M.A., Grinblat, Y.
ID
ZDB-PUB-161001-5
Date
2016
Source
Scientific Reports   6: 34437 (Journal)
Registered Authors
Grinblat, Yevgenya, Nyholm, Molly Wagner, TeSlaa, Jessica Joy, Wolman, Marc
Keywords
Developmental neurogenesis, Mechanisms of disease
MeSH Terms
none
PubMed
27687975 Full text @ Sci. Rep.
Abstract
Cilia are cell surface organelles with key roles in a range of cellular processes, including generation of fluid flow by motile cilia. The axonemes of motile cilia and immotile kinocilia contain 9 peripheral microtubule doublets, a central microtubule pair, and 9 connecting radial spokes. Aberrant radial spoke components RSPH1, 3, 4a and 9 have been linked with primary ciliary dyskinesia (PCD), a disorder characterized by ciliary dysmotility; yet, radial spoke functions remain unclear. Here we show that zebrafish Rsph9 is expressed in cells bearing motile cilia and kinocilia, and localizes to both 9 + 2 and 9 + 0 ciliary axonemes. Using CRISPR mutagenesis, we show that rsph9 is required for motility of presumptive 9 + 2 olfactory cilia and, unexpectedly, 9 + 0 neural cilia. rsph9 is also required for the structural integrity of 9 + 2 and 9 + 0 ciliary axonemes. rsph9 mutant larvae exhibit reduced initiation of the acoustic startle response consistent with hearing impairment, suggesting a novel role for Rsph9 in the kinocilia of the inner ear and/or lateral line neuromasts. These data identify novel roles for Rsph9 in 9 + 0 motile cilia and in sensory kinocilia, and establish a useful zebrafish PCD model.
Genes / Markers
Figures
Figure Gallery (12 images) / 2
Show all Figures
Expression
Phenotype
Mutations / Transgenics
Allele Construct Type Affected Genomic Region
hsc5TgTransgenic Insertion
    uw208
      Small Deletion
      uw212
        Indel
        1 - 3 of 3
        Show
        Human Disease / Model
        Human Disease Fish Conditions Evidence
        primary ciliary dyskinesiaTAS
        1 - 1 of 1
        Show
        Sequence Targeting Reagents
        Target Reagent Reagent Type
        rsph9CRISPR1-rsph9CRISPR
        rsph9MO3-rsph9MRPHLNO
        1 - 2 of 2
        Show
        Fish
        Antibodies
        Name Type Antigen Genes Isotypes Host Organism
        Ab1-actbmonoclonal
          IgG1Mouse
          Ab1-rsph9polyclonalRabbit
          Ab1-tubamonoclonal
            IgG2bMouse
            1 - 3 of 3
            Show
            Orthology
            Gene Orthology
            rsph9
            1 - 1 of 1
            Show
            Engineered Foreign Genes
            Marker Marker Type Name
            GFPEFGGFP
            1 - 1 of 1
            Show
            Mapping
            No data available