Published in Development on October 04, 2006
Dyskinesia, Heterotaxy and Congenital Heart Disease | NCT00608556
Ciliopathies. N Engl J Med (2011) 6.28
Identification of ciliary localization sequences within the third intracellular loop of G protein-coupled receptors. Mol Biol Cell (2008) 2.73
Vangl2 directs the posterior tilting and asymmetric localization of motile primary cilia. Nat Cell Biol (2010) 2.68
Nephronophthisis: disease mechanisms of a ciliopathy. J Am Soc Nephrol (2008) 2.66
Intraflagellar transport motors in cilia: moving along the cell's antenna. J Cell Biol (2008) 2.44
Sensory signaling-dependent remodeling of olfactory cilia architecture in C. elegans. Dev Cell (2008) 2.26
BBS6, BBS10, and BBS12 form a complex with CCT/TRiC family chaperonins and mediate BBSome assembly. Proc Natl Acad Sci U S A (2010) 2.09
A role for primary cilia in glutamatergic synaptic integration of adult-born neurons. Nat Neurosci (2012) 1.90
Cystin localizes to primary cilia via membrane microdomains and a targeting motif. J Am Soc Nephrol (2009) 1.79
Zebrafish mutations affecting cilia motility share similar cystic phenotypes and suggest a mechanism of cyst formation that differs from pkd2 morphants. Dev Biol (2007) 1.74
Increased postoperative and respiratory complications in patients with congenital heart disease associated with heterotaxy. J Thorac Cardiovasc Surg (2010) 1.74
A hitchhiker's guide to mechanobiology. Dev Cell (2011) 1.72
Intraflagellar transport particle size scales inversely with flagellar length: revisiting the balance-point length control model. J Cell Biol (2009) 1.62
Ciliary abnormalities due to defects in the retrograde transport protein DYNC2H1 in short-rib polydactyly syndrome. Am J Hum Genet (2009) 1.59
NEK1 mutations cause short-rib polydactyly syndrome type majewski. Am J Hum Genet (2011) 1.56
Morphogenesis of the node and notochord: the cellular basis for the establishment and maintenance of left-right asymmetry in the mouse. Dev Dyn (2008) 1.50
Convergent extension movements and ciliary function are mediated by ofd1, a zebrafish orthologue of the human oral-facial-digital type 1 syndrome gene. Hum Mol Genet (2008) 1.46
The Talpid3 gene (KIAA0586) encodes a centrosomal protein that is essential for primary cilia formation. Development (2009) 1.45
Ab ovo or de novo? Mechanisms of centriole duplication. Mol Cells (2009) 1.43
Drosophila bld10 is a centriolar protein that regulates centriole, basal body, and motile cilium assembly. Mol Biol Cell (2009) 1.40
Building it up and taking it down: the regulation of vertebrate ciliogenesis. Dev Dyn (2008) 1.38
LRRC50, a conserved ciliary protein implicated in polycystic kidney disease. J Am Soc Nephrol (2008) 1.38
Nephrocystin-1 and nephrocystin-4 are required for epithelial morphogenesis and associate with PALS1/PATJ and Par6. Hum Mol Genet (2009) 1.37
Lack of primary cilia primes shear-induced endothelial-to-mesenchymal transition. Circ Res (2011) 1.31
Mutations in zebrafish leucine-rich repeat-containing six-like affect cilia motility and result in pronephric cysts, but have variable effects on left-right patterning. Development (2009) 1.31
The dynein regulatory complex is required for ciliary motility and otolith biogenesis in the inner ear. Nature (2008) 1.31
Direct interactions of intraflagellar transport complex B proteins IFT88, IFT52, and IFT46. J Biol Chem (2010) 1.31
Intraflagellar transport protein 172 is essential for primary cilia formation and plays a vital role in patterning the mammalian brain. Dev Biol (2008) 1.30
A primary cilia-dependent etiology for midline facial disorders. Hum Mol Genet (2010) 1.29
Identification of novel regulatory factor X (RFX) target genes by comparative genomics in Drosophila species. Genome Biol (2007) 1.28
Primary ciliary dyskinesia in mice lacking the novel ciliary protein Pcdp1. Mol Cell Biol (2007) 1.26
Functional interactions between the ciliopathy-associated Meckel syndrome 1 (MKS1) protein and two novel MKS1-related (MKSR) proteins. J Cell Sci (2009) 1.26
Functional characterization of the OFD1 protein reveals a nuclear localization and physical interaction with subunits of a chromatin remodeling complex. Mol Biol Cell (2007) 1.25
C2cd3 is required for cilia formation and Hedgehog signaling in mouse. Development (2008) 1.18
Glis3 is associated with primary cilia and Wwtr1/TAZ and implicated in polycystic kidney disease. Mol Cell Biol (2009) 1.18
A mouse model for Meckel syndrome type 3. J Am Soc Nephrol (2009) 1.16
Cep70 and Cep131 contribute to ciliogenesis in zebrafish embryos. BMC Cell Biol (2009) 1.14
The dynamic cilium in human diseases. Pathogenetics (2009) 1.13
Centrosomal localization of Diversin and its relevance to Wnt signaling. J Cell Sci (2009) 1.12
NPHP4 is necessary for normal photoreceptor ribbon synapse maintenance and outer segment formation, and for sperm development. Hum Mol Genet (2010) 1.09
Fish and frogs: models for vertebrate cilia signaling. Front Biosci (2008) 1.08
Polycystin-2 takes different routes to the somatic and ciliary plasma membrane. J Cell Biol (2011) 1.07
Cilia involvement in patterning and maintenance of the skeleton. Curr Top Dev Biol (2008) 1.06
Gli-similar (Glis) Krüppel-like zinc finger proteins: insights into their physiological functions and critical roles in neonatal diabetes and cystic renal disease. Histol Histopathol (2010) 1.04
Kidney-specific inactivation of Ofd1 leads to renal cystic disease associated with upregulation of the mTOR pathway. Hum Mol Genet (2010) 1.02
Intraflagellar transport at a glance. J Cell Sci (2009) 1.02
Oda16/Wdr69 is essential for axonemal dynein assembly and ciliary motility during zebrafish embryogenesis. Dev Dyn (2010) 1.01
Regulation of a heterodimeric kinesin-2 through an unprocessive motor domain that is turned processive by its partner. Proc Natl Acad Sci U S A (2010) 1.01
RFX2 is essential in the ciliated organ of asymmetry and an RFX2 transgene identifies a population of ciliated cells sufficient for fluid flow. Dev Biol (2011) 1.01
Total internal reflection fluorescence (TIRF) microscopy of Chlamydomonas flagella. Methods Cell Biol (2009) 0.99
Depletion of plasma membrane PtdIns(4,5)P2 reveals essential roles for phosphoinositides in flagellar biogenesis. J Cell Sci (2008) 0.97
Gli-similar proteins: their mechanisms of action, physiological functions, and roles in disease. Vitam Horm (2012) 0.95
The polarity protein Pard3 is required for centrosome positioning during neurulation. Dev Biol (2010) 0.95
The ciliogenic protein Oral-Facial-Digital 1 regulates the neuronal differentiation of embryonic stem cells. Stem Cells Dev (2010) 0.94
APOBEC2, a selective inhibitor of TGFβ signaling, regulates left-right axis specification during early embryogenesis. Dev Biol (2010) 0.93
The Cdc14B phosphatase contributes to ciliogenesis in zebrafish. Development (2011) 0.91
Molecular Biomechanics: The Molecular Basis of How Forces Regulate Cellular Function. Mol Cell Biomech (2010) 0.91
The role of hair cells, cilia and ciliary motility in otolith formation in the zebrafish otic vesicle. Development (2012) 0.90
Fuz regulates craniofacial development through tissue specific responses to signaling factors. PLoS One (2011) 0.90
Zic3 is required in the migrating primitive streak for node morphogenesis and left-right patterning. Hum Mol Genet (2013) 0.89
Analysis of the orientation of primary cilia in growth plate cartilage: a mathematical method based on multiphoton microscopical images. J Struct Biol (2006) 0.89
Loss of SPEF2 function in mice results in spermatogenesis defects and primary ciliary dyskinesia. Biol Reprod (2011) 0.89
Roles of planar cell polarity pathways in the development of neural [correction of neutral] tube defects. J Biomed Sci (2011) 0.88
Mating behavior, male sensory cilia, and polycystins in Caenorhabditis elegans. Semin Cell Dev Biol (2014) 0.88
The intraflagellar transport protein IFT80 is required for cilia formation and osteogenesis. Bone (2012) 0.88
KIF3A, a cilia structural gene on chromosome 5q31, and its polymorphisms show an association with aspirin hypersensitivity in asthma. J Clin Immunol (2010) 0.86
Ciliogenesis in normal human kidney development and post-natal life. Pediatr Nephrol (2011) 0.85
Kif3a deficiency reverses the skeletal abnormalities in Pkd1 deficient mice by restoring the balance between osteogenesis and adipogenesis. PLoS One (2010) 0.84
Sox17 and chordin are required for formation of Kupffer's vesicle and left-right asymmetry determination in zebrafish. Dev Dyn (2010) 0.84
Salient features of the ciliated organ of asymmetry. Bioarchitecture (2014) 0.84
NPHP4 variants are associated with pleiotropic heart malformations. Circ Res (2012) 0.83
The Meckel syndrome protein meckelin (TMEM67) is a key regulator of cilia function but is not required for tissue planar polarity. Hum Mol Genet (2013) 0.83
Transmembrane protein OSTA-1 shapes sensory cilia morphology via regulation of intracellular membrane trafficking in C. elegans. Development (2013) 0.82
Polycystic kidney disease in the medaka (Oryzias latipes) pc mutant caused by a mutation in the Gli-Similar3 (glis3) gene. PLoS One (2009) 0.82
TRANSCRIPTION FACTOR GLI-SIMILAR 3 (GLIS3): IMPLICATIONS FOR THE DEVELOPMENT OF CONGENITAL HYPOTHYROIDISM. J Endocrinol Diabetes Obes (2014) 0.81
Spatial distribution of niche and stem cells in ex vivo human limbal cultures. Stem Cells Transl Med (2014) 0.81
Sept7b is essential for pronephric function and development of left-right asymmetry in zebrafish embryogenesis. J Cell Sci (2014) 0.81
Axonemal positioning and orientation in three-dimensional space for primary cilia: what is known, what is assumed, and what needs clarification. Dev Dyn (2011) 0.80
Geometry-specific heterogeneity of the apparent diffusion rate of materials inside sperm cells. Biophys J (2010) 0.80
Interkinetic nuclear migration generates and opposes ventricular-zone crowding: insight into tissue mechanics. Front Cell Neurosci (2015) 0.80
The membrane proteome of sensory cilia to the depth of olfactory receptors. Mol Cell Proteomics (2014) 0.80
Topographically-patterned porous membranes in a microfluidic device as an in vitro model of renal reabsorptive barriers. Lab Chip (2013) 0.80
The cilia-regulated proteasome and its role in the development of ciliopathies and cancer. Cilia (2016) 0.80
Regional selection acting on the OFD1 gene family. PLoS One (2011) 0.79
Functional redundancy between Cdc14 phosphatases in zebrafish ciliogenesis. Dev Dyn (2012) 0.79
Chibby functions to preserve normal ciliary morphology through the regulation of intraflagellar transport in airway ciliated cells. Cell Cycle (2015) 0.78
Functional Analysis of KIF3A and KIF3B during Spermiogenesis of Chinese Mitten Crab Eriocheir sinensis. PLoS One (2014) 0.78
Intracellular and extracellular forces drive primary cilia movement. Proc Natl Acad Sci U S A (2015) 0.77
Congenital Heart Disease Genetics Uncovers Context-Dependent Organization and Function of Nucleoporins at Cilia. Dev Cell (2016) 0.77
Antennas of organ morphogenesis: the roles of cilia in vertebrate kidney development. Genesis (2016) 0.76
Fluid shear stress-induced TGF-β/ALK5 signaling in renal epithelial cells is modulated by MEK1/2. Cell Mol Life Sci (2017) 0.75
The evolving spectrum of ciliopathies and respiratory disease. Curr Opin Pediatr (2016) 0.75
Craniofacial ciliopathies: An expanding oral disease spectrum - a review of literature and a case report. Indian J Dent (2016) 0.75
A Point Mutation in p190A RhoGAP Affects Ciliogenesis and Leads to Glomerulocystic Kidney Defects. PLoS Genet (2016) 0.75
Protein quality control by Rer1p in the early secretory pathway: from mechanism to implication in Alzheimer's disease. Alzheimers Res Ther (2013) 0.75
Nephrotic syndrome in a patient with situs inversus totalis. Clin Exp Nephrol (2008) 0.75
Acromelia-oligodontia syndrome. Clin Case Rep (2017) 0.75
The Tol2kit: a multisite gateway-based construction kit for Tol2 transposon transgenesis constructs. Dev Dyn (2007) 8.86
Kupffer's vesicle is a ciliated organ of asymmetry in the zebrafish embryo that initiates left-right development of the brain, heart and gut. Development (2005) 4.24
Mechanical force mobilizes zyxin from focal adhesions to actin filaments and regulates cytoskeletal reinforcement. J Cell Biol (2005) 3.13
Conserved function for embryonic nodal cilia. Nature (2002) 2.56
FGF signalling during embryo development regulates cilia length in diverse epithelia. Nature (2009) 1.91
Polaris and Polycystin-2 in dorsal forerunner cells and Kupffer's vesicle are required for specification of the zebrafish left-right axis. Dev Biol (2005) 1.69
The T box transcription factor no tail in ciliated cells controls zebrafish left-right asymmetry. Curr Biol (2004) 1.58
Protein phosphatase 1 regulates assembly and function of the beta-catenin degradation complex. EMBO J (2007) 1.55
Lefty-dependent inhibition of Nodal- and Wnt-responsive organizer gene expression is essential for normal gastrulation. Curr Biol (2002) 1.42
Regulation of casein kinase I epsilon activity by Wnt signaling. J Biol Chem (2004) 1.40
MMAPPR: mutation mapping analysis pipeline for pooled RNA-seq. Genome Res (2013) 1.39
Two T-box genes play independent and cooperative roles to regulate morphogenesis of ciliated Kupffer's vesicle in zebrafish. Dev Biol (2007) 1.36
Adenomatous polyposis coli control of retinoic acid biosynthesis is critical for zebrafish intestinal development and differentiation. J Biol Chem (2004) 1.31
Quantification of stretch-induced cytoskeletal remodeling in vascular endothelial cells by image processing. Cytometry A (2003) 1.28
Baf60c is a nuclear Notch signaling component required for the establishment of left-right asymmetry. Proc Natl Acad Sci U S A (2007) 1.26
A rapid and efficient method of genotyping zebrafish mutants. Dev Dyn (2009) 1.23
Ectodermal syndecan-2 mediates left-right axis formation in migrating mesoderm as a cell-nonautonomous Vg1 cofactor. Dev Cell (2002) 1.23
A Wnt-CKIvarepsilon-Rap1 pathway regulates gastrulation by modulating SIPA1L1, a Rap GTPase activating protein. Dev Cell (2007) 1.22
Heparan sulfate core proteins in cell-cell signaling. Annu Rev Genet (2003) 1.16
Extra-embryonic syndecan 2 regulates organ primordia migration and fibrillogenesis throughout the zebrafish embryo. Development (2009) 1.13
Genetics of human laterality disorders: insights from vertebrate model systems. Annu Rev Genomics Hum Genet (2003) 1.12
PKCgamma regulates syndecan-2 inside-out signaling during xenopus left-right development. Cell (2002) 1.07
Poly peak parser: Method and software for identification of unknown indels using sanger sequencing of polymerase chain reaction products. Dev Dyn (2014) 1.07
The Rho kinase Rock2b establishes anteroposterior asymmetry of the ciliated Kupffer's vesicle in zebrafish. Development (2010) 1.06
Combinatorial expression patterns of heparan sulfate sulfotransferases in zebrafish: I. The 3-O-sulfotransferase family. Dev Dyn (2006) 1.05
Cardiac neural crest contributes to cardiomyogenesis in zebrafish. Dev Biol (2003) 1.05
Transient axonal glycoprotein-1 (TAG-1) and laminin-alpha1 regulate dynamic growth cone behaviors and initial axon direction in vivo. Neural Dev (2008) 1.01
RFX2 is essential in the ciliated organ of asymmetry and an RFX2 transgene identifies a population of ciliated cells sufficient for fluid flow. Dev Biol (2011) 1.01
The left-right determinant inversin has highly conserved ankyrin repeat and IQ domains and interacts with calmodulin. Hum Genet (2002) 1.00
Genetic modeling of Li-Fraumeni syndrome in zebrafish. Dis Model Mech (2010) 0.95
The zebrafish retinol dehydrogenase, rdh1l, is essential for intestinal development and is regulated by the tumor suppressor adenomatous polyposis coli. J Biol Chem (2005) 0.92
A family-based paradigm to identify candidate chromosomal regions for isolated congenital diaphragmatic hernia. Am J Med Genet A (2012) 0.91
Initiation and propagation of posterior to anterior (PA) waves in zebrafish left-right development. Dev Dyn (2008) 0.91
Combinatorial expression patterns of heparan sulfate sulfotransferases in zebrafish: III. 2-O-sulfotransferase and C5-epimerases. Dev Dyn (2007) 0.91
ALK4 functions as a receptor for multiple TGF beta-related ligands to regulate left-right axis determination and mesoderm induction in Xenopus. Dev Biol (2004) 0.90
Combinatorial expression patterns of heparan sulfate sulfotransferases in zebrafish: II. The 6-O-sulfotransferase family. Dev Dyn (2006) 0.90
Differential roles for 3-OSTs in the regulation of cilia length and motility. Development (2013) 0.87
Dual roles for adenomatous polyposis coli in regulating retinoic acid biosynthesis and Wnt during ocular development. Proc Natl Acad Sci U S A (2006) 0.87
Disease-associated casein kinase I delta mutation may promote adenomatous polyps formation via a Wnt/beta-catenin independent mechanism. Int J Cancer (2007) 0.85
Lethal giant larvae 2 regulates development of the ciliated organ Kupffer's vesicle. Development (2013) 0.85
Hadp1, a newly identified pleckstrin homology domain protein, is required for cardiac contractility in zebrafish. Dis Model Mech (2011) 0.85
Rer1p maintains ciliary length and signaling by regulating γ-secretase activity and Foxj1a levels. J Cell Biol (2013) 0.84
2-O-sulfotransferase regulates Wnt signaling, cell adhesion and cell cycle during zebrafish epiboly. Development (2012) 0.83
Nodal signaling: CrypticLefty mechanism of antagonism decoded. Curr Biol (2004) 0.83
14-3-3ε plays a role in cardiac ventricular compaction by regulating the cardiomyocyte cell cycle. Mol Cell Biol (2012) 0.83
Semaphorin3D regulates invasion of cardiac neural crest cells into the primary heart field. Dev Biol (2006) 0.83
Genomic characterization and expression analysis of the first nonmammalian renin genes from zebrafish and pufferfish. Physiol Genomics (2004) 0.83
Analysis of gene function and visualization of cilia-generated fluid flow in Kupffer's vesicle. J Vis Exp (2013) 0.81
3-OST-7 regulates BMP-dependent cardiac contraction. PLoS Biol (2013) 0.81
Genome-wide analysis reveals the unique stem cell identity of human amniocytes. PLoS One (2013) 0.80
Sdc2 and Tbx16 regulate Fgf2-dependent epithelial cell morphogenesis in the ciliated organ of asymmetry. Development (2013) 0.80
14-3-3ε gene variants in a Japanese patient with left ventricular noncompaction and hypoplasia of the corpus callosum. Gene (2012) 0.80
Dvr1 transfers left-right asymmetric signals from Kupffer's vesicle to lateral plate mesoderm in zebrafish. Dev Biol (2013) 0.78
A one-step miniprep for the isolation of plasmid DNA and lambda phage particles. PLoS One (2011) 0.76
Coordinating the development of bilateral symmetry and left-right asymmetry. Semin Cell Dev Biol (2009) 0.76
Micro-Computed Tomography for the Quantitative 3-Dimensional Assessment of the Compact Myocardium in the Mouse Embryo. Circ J (2016) 0.76
The COXes of Danio: from mechanistic model to experimental therapeutics. Proc Natl Acad Sci U S A (2002) 0.75