Cilia

• 

  Subject Areas on Research

  • A Mec17-Myosin II Effector Axis Coordinates Microtubule Acetylation and Actin Dynamics to Control Primary Cilium Biogenesis. 
  • A beta-adrenergic receptor kinase-like enzyme is involved in olfactory signal termination. 
  • A novel role for primary cilia in airway remodeling. 
  • A proteomic analysis of human cilia: identification of novel components. 
  • A transition zone complex regulates mammalian ciliogenesis and ciliary membrane composition. 
  • ARMC4 mutations cause primary ciliary dyskinesia with randomization of left/right body asymmetry. 
  • Activation by odorants of a multistate cation channel from olfactory cilia. 
  • An essential role for DYF-11/MIP-T3 in assembling functional intraflagellar transport complexes. 
  • An organelle-specific protein landscape identifies novel diseases and molecular mechanisms. 
  • Ankyrin-G promotes cyclic nucleotide-gated channel transport to rod photoreceptor sensory cilia. 
  • BBS4 regulates the expression and secretion of FSTL1, a protein that participates in ciliogenesis and the differentiation of 3T3-L1. 
  • Bardet-Biedl syndrome-associated small GTPase ARL6 (BBS3) functions at or near the ciliary gate and modulates Wnt signaling. 
  • Basal body dysfunction is a likely cause of pleiotropic Bardet-Biedl syndrome. 
  • Beta-arrestin-mediated localization of smoothened to the primary cilium. 
  • CC2D2A is mutated in Joubert syndrome and interacts with the ciliopathy-associated basal body protein CEP290. 
  • CCDC39 is required for assembly of inner dynein arms and the dynein regulatory complex and for normal ciliary motility in humans and dogs. 
  • Cilia in vertebrate development and disease. 
  • Cilia loss sensitizes cells to transformation by activating the mevalonate pathway. 
  • Ciliary function and Wnt signal modulation. 
  • Ciliary proteins and exencephaly. 
  • Ciliated epithelial cell lifespan in the mouse trachea and lung. 
  • Ciliopathies. 
  • Comparative genomics identifies a flagellar and basal body proteome that includes the BBS5 human disease gene. 
  • Conditional stabilization of beta-catenin expands the pool of lung stem cells. 
  • Context-dependent regulation of Wnt signaling through the primary cilium. 
  • Contribution of hedgehog signaling to the establishment of left-right asymmetry in the sea urchin. 
  • Crescerin uses a TOG domain array to regulate microtubules in the primary cilium. 
  • DCDC2 mutations cause a renal-hepatic ciliopathy by disrupting Wnt signaling. 
  • DNAH11 Localization in the Proximal Region of Respiratory Cilia Defines Distinct Outer Dynein Arm Complexes. 
  • Development of a tracheal implant xenograft model to expose human bronchial epithelial cells to toxic gases. 
  • Diagnostic yield of nasal scrape biopsies in primary ciliary dyskinesia: a multicenter experience. 
  • Discordant organ laterality in monozygotic twins with primary ciliary dyskinesia. 
  • Disruption of Bardet-Biedl syndrome ciliary proteins perturbs planar cell polarity in vertebrates. 
  • Dissecting intraflagellar transport, one molecule at a time. 
  • Dual SMAD Signaling Inhibition Enables Long-Term Expansion of Diverse Epithelial Basal Cells. 
  • Effect of high oxygen tension on mucociliary function. 
  • Evolutionarily assembled cis-regulatory module at a human ciliopathy locus. 
  • Exome capture reveals ZNF423 and CEP164 mutations, linking renal ciliopathies to DNA damage response signaling. 
  • Expanding CEP290 mutational spectrum in ciliopathies. 
  • Flagellar motility requires the cAMP-dependent phosphorylation of a heat-stable NP-40-soluble 56 kd protein, axokinin. 
  • Functional interactions between the ciliopathy-associated Meckel syndrome 1 (MKS1) protein and two novel MKS1-related (MKSR) proteins. 
  • Gene expression and larval evolution: changing roles of distal-less and orthodenticle in echinoderm larvae. 
  • Gene therapy rescues cilia defects and restores olfactory function in a mammalian ciliopathy model. 
  • Genetic causes of bronchiectasis: primary ciliary dyskinesia. 
  • Growth Arrest Specific 8 (Gas8) and G protein-coupled receptor kinase 2 (GRK2) cooperate in the control of Smoothened signaling. 
  • Hedgehog signaling requires motile cilia in the sea urchin. 
  • IFT46 plays an essential role in cilia development. 
  • IL-6/STAT3 promotes regeneration of airway ciliated cells from basal stem cells. 
  • Individuals with mutations in XPNPEP3, which encodes a mitochondrial protein, develop a nephronophthisis-like nephropathy. 
  • Joubert Syndrome in French Canadians and Identification of Mutations in CEP104. 
  • KIF7 mutations cause fetal hydrolethalus and acrocallosal syndromes. 
  • Lack of effect of ammonium glycyrrhizinate on the morphology of ovine nasal mucosa in vitro. 
  • Life without centrioles: cilia in the spotlight. 
  • Living in a physical world X. Pumping fluids through conduits. 
  • Localization of ATPase in cilia of rabbit trachea. 
  • Loss of Arf4 causes severe degeneration of the exocrine pancreas but not cystic kidney disease or retinal degeneration. 
  • Loss of BBS proteins causes anosmia in humans and defects in olfactory cilia structure and function in the mouse. 
  • Loss of Bardet-Biedl syndrome protein-8 (BBS8) perturbs olfactory function, protein localization, and axon targeting. 
  • Loss of C. elegans BBS-7 and BBS-8 protein function results in cilia defects and compromised intraflagellar transport. 
  • Lung development and repair: contribution of the ciliated lineage. 
  • Lung mucociliary function in man: interdependence of bronchial and tracheal mucus transport velocities with lung clearance in bronchial asthma and healthy subjects. 
  • MKKS/BBS6, a divergent chaperonin-like protein linked to the obesity disorder Bardet-Biedl syndrome, is a novel centrosomal component required for cytokinesis. 
  • Mechanistic insights into Bardet-Biedl syndrome, a model ciliopathy. 
  • Metabolic regulation and energy homeostasis through the primary Cilium. 
  • Mucin is produced by clara cells in the proximal airways of antigen-challenged mice. 
  • Mutation analysis of 18 nephronophthisis associated ciliopathy disease genes using a DNA pooling and next generation sequencing strategy. 
  • Mutations in CSPP1, encoding a core centrosomal protein, cause a range of ciliopathy phenotypes in humans. 
  • Mutations in INVS encoding inversin cause nephronophthisis type 2, linking renal cystic disease to the function of primary cilia and left-right axis determination. 
  • Mutations in RSPH1 cause primary ciliary dyskinesia with a unique clinical and ciliary phenotype. 
  • Mutations in TMEM216 perturb ciliogenesis and cause Joubert, Meckel and related syndromes. 
  • Mutations in a member of the Ras superfamily of small GTP-binding proteins causes Bardet-Biedl syndrome. 
  • Mutations in the cilia gene ARL13B lead to the classical form of Joubert syndrome. 
  • Nde1-mediated inhibition of ciliogenesis affects cell cycle re-entry. 
  • Nephrocystin-3 is required for ciliary function in zebrafish embryos. 
  • Nephronophthisis-associated ciliopathies. 
  • Olfactory desensitization requires membrane targeting of receptor kinase mediated by beta gamma-subunits of heterotrimeric G proteins. 
  • Parent stem cells can serve as niches for their daughter cells. 
  • Photoreceptor discs form through peripherin-dependent suppression of ciliary ectosome release. 
  • Pitchfork regulates primary cilia disassembly and left-right asymmetry. 
  • Planar cell polarity acts through septins to control collective cell movement and ciliogenesis. 
  • Presence and indirect immunofluorescent localization of calmodulin in Paramecium tetraurelia. 
  • Primary Cilia Signaling Shapes the Development of Interneuronal Connectivity. 
  • Primary cilia attenuate hedgehog signalling in neoplastic chondrocytes. 
  • Primary cilium-dependent and -independent Hedgehog signaling inhibits p16(INK4A). 
  • Proteomic Analysis of Primary Human Airway Epithelial Cells Exposed to the Respiratory Toxicant Diacetyl. 
  • RPGR isoforms in photoreceptor connecting cilia and the transitional zone of motile cilia. 
  • Regulation of hedgehog signaling by Myc-interacting zinc finger protein 1, Miz1. 
  • Scanning electron microscopic study of the airways in normal children and in patients with cystic fibrosis and other lung diseases. 
  • Social feeding in Caenorhabditis elegans is induced by neurons that detect aversive stimuli. 
  • Spinocerebellar ataxia type 11-associated alleles of Ttbk2 dominantly interfere with ciliogenesis and cilium stability. 
  • TMEM231, mutated in orofaciodigital and Meckel syndromes, organizes the ciliary transition zone. 
  • TMEM237 is mutated in individuals with a Joubert syndrome related disorder and expands the role of the TMEM family at the ciliary transition zone. 
  • TRPV4 channel participates in receptor-operated calcium entry and ciliary beat frequency regulation in mouse airway epithelial cells. 
  • The Bardet-Biedl syndrome-related protein CCDC28B modulates mTORC2 function and interacts with SIN1 to control cilia length independently of the mTOR complex. 
  • The Meckel-Gruber Syndrome proteins MKS1 and meckelin interact and are required for primary cilium formation. 
  • The actin nucleator Cordon-bleu is required for development of motile cilia in zebrafish. 
  • The centrosome in human genetic disease. 
  • The ciliary proteome database: an integrated community resource for the genetic and functional dissection of cilia. 
  • The ciliopathies: a transitional model into systems biology of human genetic disease. 
  • The complexity of the cilium: spatiotemporal diversity of an ancient organelle. 
  • The effect of ventilation and oxygen on mucociliary clearance and alveolar stability. 
  • The emerging complexity of the vertebrate cilium: new functional roles for an ancient organelle. 
  • The kinetochore protein, CENPF, is mutated in human ciliopathy and microcephaly phenotypes. 
  • The localization of succinic dehydrogenase, cytochrome oxidase and adenosine triphosphatase in ciliated respiratory epithelium. 
  • The molecular identities of the Caenorhabditis elegans intraflagellar transport genes dyf-6, daf-10 and osm-1. 
  • The pathogenesis of nontypable Haemophilus influenzae otitis media. 
  • The prevalence of abnormal cilia in normal pediatric lungs. 
  • The primary cilium as a Hedgehog signal transduction machine. 
  • The primary cilium: a signalling centre during vertebrate development. 
  • The retinitis pigmentosa protein RP2 interacts with polycystin 2 and regulates cilia-mediated vertebrate development. 
  • The spinocerebellar ataxia-associated gene Tau tubulin kinase 2 controls the initiation of ciliogenesis. 
  • The vertebrate primary cilium in development, homeostasis, and disease. 
  • Uncovering inherent cellular plasticity of multiciliated ependyma leading to ventricular wall transformation and hydrocephalus. 
  • ZMYND10 is mutated in primary ciliary dyskinesia and interacts with LRRC6. 
  • Zebrafish assays of ciliopathies. 
• 

  Keywords of People

  • Bennett, Vann, George B. Geller Professor for Research in Molecular Biology in the School of Medicine, Duke Cancer Institute