56 results found
    1. Developmental Biology

    Dysregulation of sonic hedgehog signaling causes hearing loss in ciliopathy mouse models

    Kyeong-Hye Moon et al.
    Developmental defects of the cochlea caused by dysregulation of sonic hedgehog signaling are the potential etiology for hearing loss in a group of ciliopathies with defective ciliogenesis.
    1. Cell Biology
    2. Developmental Biology

    Dzip1 and Fam92 form a ciliary transition zone complex with cell type specific roles in Drosophila

    Jean-André Lapart et al.
    Genetic analyses in Drosophila establish the functional hierarchy of the ciliary transition zone module, Dzip1/Fam92/Cby, and reveal tissue specific variations in basal body anchoring pathways in fly ciliated tissues.
    1. Cell Biology
    2. Neuroscience

    Ciliary neuropeptidergic signaling dynamically regulates excitatory synapses in postnatal neocortical pyramidal neurons

    Lauren Tereshko et al.
    Neuropeptidergic signaling via cilia-localized receptors dynamically regulates excitatory synaptic drive in postnatal rat neocortical pyramidal neurons.
    1. Cell Biology

    A novel Cep120-dependent mechanism inhibits centriole maturation in quiescent cells

    Ewelina Betleja et al.
    Cells have evolved a mechanism that actively regulates centriole maturation during quiescence.
    1. Developmental Biology

    Functional genome-wide siRNA screen identifies KIAA0586 as mutated in Joubert syndrome

    Susanne Roosing et al.
    A supervised learning approach on a high-content genome-wide siRNA screen has identified 591 likely candidates for ciliopathies and facilitated in the discovery of KIAA0586 mutations in individuals with Joubert syndrome.
    1. Developmental Biology

    Primary cilia deficiency in neural crest cells models anterior segment dysgenesis in mouse

    Céline Portal et al.
    Primary cilia of neural crest-derived cells mediate Indian hedgehog-induced signal transduction in the periocular mesenchyme and are required for normal anterior segment development.
    1. Developmental Biology

    TALPID3 controls centrosome and cell polarity and the human ortholog KIAA0586 is mutated in Joubert syndrome (JBTS23)

    Louise A Stephen et al.
    Mutations in KIAA0586 (TALPID3) cause a severe ciliopathy called Joubert syndrome that affects organ, cell and centrosome polarity.
    1. Cell Biology
    2. Developmental Biology

    LUZP1, a novel regulator of primary cilia and the actin cytoskeleton, is a contributing factor in Townes-Brocks Syndrome

    Laura Bozal-Basterra et al.
    LUZP1 is required for proper cilia and cytoskeleton formation.
    1. Cell Biology

    A pathway for Parkinson’s Disease LRRK2 kinase to block primary cilia and Sonic hedgehog signaling in the brain

    Herschel S Dhekne et al.
    Pathogenic LRRK2kinase requires Rab10 and RILPL1 to block primary cilia formation, shortening cilia on cholinergic neurons needed for a hedgehog driven circuit that supports dopaminergic neurons in mouse brain.
    1. Cell Biology
    2. Structural Biology and Molecular Biophysics

    Crystal structure of intraflagellar transport protein 80 reveals a homo-dimer required for ciliogenesis

    Michael Taschner et al.
    CRISPR/Cas knockout of intraflagellar transport protein 80 shows that this subunit is absolutely required for ciliogenesis, and biophysical studies reveal that this protein may dimerize the intraflagellar transport complex.

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