Published in Development on December 13, 2006
Kinesin superfamily motor proteins and intracellular transport. Nat Rev Mol Cell Biol (2009) 5.61
The vertebrate primary cilium in development, homeostasis, and disease. Cell (2009) 5.29
Cilia and developmental signaling. Annu Rev Cell Dev Biol (2007) 4.88
Disruption of intraflagellar transport in adult mice leads to obesity and slow-onset cystic kidney disease. Curr Biol (2007) 3.76
Primary cilia can both mediate and suppress Hedgehog pathway-dependent tumorigenesis. Nat Med (2009) 2.99
Ciliopathies: an expanding disease spectrum. Pediatr Nephrol (2011) 2.95
Dual and opposing roles of primary cilia in medulloblastoma development. Nat Med (2009) 2.73
Coupling between hydrodynamic forces and planar cell polarity orients mammalian motile cilia. Nat Cell Biol (2010) 2.67
Intraflagellar transport, cilia, and mammalian Hedgehog signaling: analysis in mouse embryonic fibroblasts. Dev Dyn (2008) 2.30
Primary cilia mediate mechanosensing in bone cells by a calcium-independent mechanism. Proc Natl Acad Sci U S A (2007) 2.10
Primary cilia and signaling pathways in mammalian development, health and disease. Nephron Physiol (2009) 2.09
DYNC2H1 mutations cause asphyxiating thoracic dystrophy and short rib-polydactyly syndrome, type III. Am J Hum Genet (2009) 2.03
Ofd1, a human disease gene, regulates the length and distal structure of centrioles. Dev Cell (2010) 1.94
Cilia organize ependymal planar polarity. J Neurosci (2010) 1.80
Loss of primary cilia upregulates renal hypertrophic signaling and promotes cystogenesis. J Am Soc Nephrol (2011) 1.75
Complex interactions between genes controlling trafficking in primary cilia. Nat Genet (2011) 1.65
A mouse model for Meckel syndrome reveals Mks1 is required for ciliogenesis and Hedgehog signaling. Hum Mol Genet (2009) 1.55
Architecture and function of IFT complex proteins in ciliogenesis. Differentiation (2011) 1.55
A novel protein LZTFL1 regulates ciliary trafficking of the BBSome and Smoothened. PLoS Genet (2011) 1.49
Primary cilia are not required for normal canonical Wnt signaling in the mouse embryo. PLoS One (2009) 1.44
Development of the post-natal growth plate requires intraflagellar transport proteins. Dev Biol (2007) 1.44
Acentriolar mitosis activates a p53-dependent apoptosis pathway in the mouse embryo. Proc Natl Acad Sci U S A (2014) 1.41
The Oak Ridge Polycystic Kidney mouse: modeling ciliopathies of mice and men. Dev Dyn (2008) 1.36
Primary cilia regulate Shh activity in the control of molar tooth number. Development (2009) 1.31
A primary cilia-dependent etiology for midline facial disorders. Hum Mol Genet (2010) 1.29
Photoreceptor sensory cilia and ciliopathies: focus on CEP290, RPGR and their interacting proteins. Cilia (2012) 1.27
Cilia functions in development. Curr Opin Cell Biol (2012) 1.24
Primary cilia in planar cell polarity regulation of the inner ear. Curr Top Dev Biol (2008) 1.23
An essential role for dermal primary cilia in hair follicle morphogenesis. J Invest Dermatol (2008) 1.22
Different roles for KIF17 and kinesin II in photoreceptor development and maintenance. Dev Dyn (2009) 1.22
Evc2 is a positive modulator of Hedgehog signalling that interacts with Evc at the cilia membrane and is also found in the nucleus. BMC Biol (2011) 1.21
Arl13b in primary cilia regulates the migration and placement of interneurons in the developing cerebral cortex. Dev Cell (2012) 1.21
Cartilage cell clusters. Arthritis Rheum (2010) 1.21
Primary cilium-dependent mechanosensing is mediated by adenylyl cyclase 6 and cyclic AMP in bone cells. FASEB J (2010) 1.20
Trafficking in and to the primary cilium. Cilia (2012) 1.20
How do cilia organize signalling cascades? Philos Trans R Soc Lond B Biol Sci (2014) 1.19
The relationship between sonic Hedgehog signaling, cilia, and neural tube defects. Birth Defects Res A Clin Mol Teratol (2010) 1.16
Mutations in the gene encoding IFT dynein complex component WDR34 cause Jeune asphyxiating thoracic dystrophy. Am J Hum Genet (2013) 1.16
Kinesin-2 controls development and patterning of the vertebrate skeleton by Hedgehog- and Gli3-dependent mechanisms. Dev Biol (2007) 1.15
Non-random distribution and sensory functions of primary cilia in vascular smooth muscle cells. Kidney Blood Press Res (2008) 1.13
Chondrocyte moves: clever strategies? Osteoarthritis Cartilage (2007) 1.13
The primary cilium coordinates early cardiogenesis and hedgehog signaling in cardiomyocyte differentiation. J Cell Sci (2009) 1.12
An Ift80 mouse model of short rib polydactyly syndromes shows defects in hedgehog signalling without loss or malformation of cilia. Hum Mol Genet (2011) 1.10
Mutations in Traf3ip1 reveal defects in ciliogenesis, embryonic development, and altered cell size regulation. Dev Biol (2011) 1.09
Role of the polycytin-primary cilia complex in bone development and mechanosensing. Ann N Y Acad Sci (2010) 1.07
Generation of mice with functional inactivation of talpid3, a gene first identified in chicken. Development (2011) 1.07
Cilia involvement in patterning and maintenance of the skeleton. Curr Top Dev Biol (2008) 1.06
Role of epidermal primary cilia in the homeostasis of skin and hair follicles. Development (2011) 1.05
Primary cilia: cellular sensors for the skeleton. Anat Rec (Hoboken) (2008) 1.05
Depletion of primary cilia in articular chondrocytes results in reduced Gli3 repressor to activator ratio, increased Hedgehog signaling, and symptoms of early osteoarthritis. Osteoarthritis Cartilage (2011) 1.05
Polycystin-1 regulates skeletogenesis through stimulation of the osteoblast-specific transcription factor RUNX2-II. J Biol Chem (2008) 1.03
Disruption of Kif3a in osteoblasts results in defective bone formation and osteopenia. J Cell Sci (2012) 1.01
Mechanically induced osteogenic lineage commitment of stem cells. Stem Cell Res Ther (2013) 1.00
Regulation of cochlear convergent extension by the vertebrate planar cell polarity pathway is dependent on p120-catenin. Development (2012) 1.00
Mechanosensing by the primary cilium: deletion of Kif3A reduces bone formation due to loading. PLoS One (2012) 0.99
Primary cilia are required in a unique subpopulation of neural progenitors. Proc Natl Acad Sci U S A (2014) 0.98
Co-occurrence of Joubert syndrome and Jeune asphyxiating thoracic dystrophy. Am J Med Genet A (2010) 0.96
Quantitative trait loci, genes, and polymorphisms that regulate bone mineral density in mouse. Genomics (2009) 0.96
Keeping the balance between proliferation and differentiation: the primary cilium. Curr Genomics (2011) 0.96
Osteocyte primary cilium and its role in bone mechanotransduction. Ann N Y Acad Sci (2010) 0.95
Mouse models of ciliopathies: the state of the art. Dis Model Mech (2012) 0.94
A high-fat diet regulates gastrin and acid secretion through primary cilia. FASEB J (2012) 0.93
Kif3a interacts with Dynactin subunit p150 Glued to organize centriole subdistal appendages. EMBO J (2013) 0.93
The buccohypophyseal canal is an ancestral vertebrate trait maintained by modulation in sonic hedgehog signaling. BMC Biol (2013) 0.93
Mouse models for dissecting vertebrate planar cell polarity signaling in the inner ear. Brain Res (2009) 0.92
Ift88 regulates Hedgehog signaling, Sfrp5 expression, and β-catenin activity in post-natal growth plate. J Orthop Res (2012) 0.90
Cartilage abnormalities associated with defects of chondrocytic primary cilia in Bardet-Biedl syndrome mutant mice. J Orthop Res (2009) 0.90
Function and regulation of primary cilia and intraflagellar transport proteins in the skeleton. Ann N Y Acad Sci (2014) 0.90
Primary cilia modulate Ihh signal transduction in response to hydrostatic loading of growth plate chondrocytes. Bone (2011) 0.90
The primary cilium as a dual sensor of mechanochemical signals in chondrocytes. Cell Mol Life Sci (2012) 0.89
A role for the vesicle-associated tubulin binding protein ARL6 (BBS3) in flagellum extension in Trypanosoma brucei. Biochim Biophys Acta (2012) 0.89
The intraflagellar transport protein IFT80 is required for cilia formation and osteogenesis. Bone (2012) 0.88
Forward genetics uncovers Transmembrane protein 107 as a novel factor required for ciliogenesis and Sonic hedgehog signaling. Dev Biol (2012) 0.87
The ciliary protein Ftm is required for ventricular wall and septal development. PLoS One (2013) 0.87
Primary cilia attenuate hedgehog signalling in neoplastic chondrocytes. Oncogene (2012) 0.87
Mechanosensory Genes Pkd1 and Pkd2 Contribute to the Planar Polarization of Brain Ventricular Epithelium. J Neurosci (2015) 0.86
Emerging role of primary cilia as mechanosensors in osteocytes. Bone (2012) 0.86
Endothelial primary cilia inhibit atherosclerosis. EMBO Rep (2016) 0.85
Hyperglycemia in the absence of cilia accelerates cystogenesis and induces renal damage. Am J Physiol Renal Physiol (2015) 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
Generation of mice with a conditional allele for Ift172. Transgenic Res (2009) 0.84
Cilia in cell signaling and human disorders. Protein Cell (2010) 0.83
The BBSome Controls Energy Homeostasis by Mediating the Transport of the Leptin Receptor to the Plasma Membrane. PLoS Genet (2016) 0.83
Effect of localization, length and orientation of chondrocytic primary cilium on murine growth plate organization. J Theor Biol (2011) 0.82
Increased Na+/H+ exchanger activity on the apical surface of a cilium-deficient cortical collecting duct principal cell model of polycystic kidney disease. Am J Physiol Cell Physiol (2012) 0.82
Primary Cilia on Horizontal Basal Cells Regulate Regeneration of the Olfactory Epithelium. J Neurosci (2015) 0.81
Cilia movement regulates expression of the Raf-1 kinase inhibitor protein. Am J Physiol Renal Physiol (2011) 0.81
Multiple essential roles for primary cilia in heart development. Cilia (2012) 0.81
IFT81, encoding an IFT-B core protein, as a very rare cause of a ciliopathy phenotype. J Med Genet (2015) 0.81
Heterotrimeric kinesin-2 (KIF3) mediates transition zone and axoneme formation of mouse photoreceptors. J Biol Chem (2015) 0.80
A mutation in the mouse ttc26 gene leads to impaired hedgehog signaling. PLoS Genet (2014) 0.80
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
Deletion of airway cilia results in noninflammatory bronchiectasis and hyperreactive airways. Am J Physiol Lung Cell Mol Physiol (2013) 0.79
Activation of the intrarenal renin-angiotensin-system in murine polycystic kidney disease. Physiol Rep (2015) 0.79
The eukaryotic flagellum makes the day: novel and unforeseen roles uncovered after post-genomics and proteomics data. Curr Protein Pept Sci (2012) 0.78
Cilia/Ift protein and motor -related bone diseases and mouse models. Front Biosci (Landmark Ed) (2015) 0.78
Quantitative peptidomics of Purkinje cell degeneration mice. PLoS One (2013) 0.78
Hippocampal and cortical primary cilia are required for aversive memory in mice. PLoS One (2014) 0.78
Ciliary IFT80 balances canonical versus non-canonical hedgehog signalling for osteoblast differentiation. Nat Commun (2016) 0.78
Primary Cilia and Mammalian Hedgehog Signaling. Cold Spring Harb Perspect Biol (2016) 0.78
A core complex of BBS proteins cooperates with the GTPase Rab8 to promote ciliary membrane biogenesis. Cell (2007) 10.75
Gli2 and Gli3 localize to cilia and require the intraflagellar transport protein polaris for processing and function. PLoS Genet (2005) 6.15
Comparative genomics identifies a flagellar and basal body proteome that includes the BBS5 human disease gene. Cell (2004) 6.10
Cilia-driven fluid flow in the zebrafish pronephros, brain and Kupffer's vesicle is required for normal organogenesis. Development (2005) 4.64
Ciliary proteins link basal body polarization to planar cell polarity regulation. Nat Genet (2007) 3.79
Disruption of intraflagellar transport in adult mice leads to obesity and slow-onset cystic kidney disease. Curr Biol (2007) 3.76
The primary cilium as a complex signaling center. Curr Biol (2009) 3.73
A BBSome subunit links ciliogenesis, microtubule stability, and acetylation. Dev Cell (2008) 3.17
MKS and NPHP modules cooperate to establish basal body/transition zone membrane associations and ciliary gate function during ciliogenesis. J Cell Biol (2011) 3.11
An incredible decade for the primary cilium: a look at a once-forgotten organelle. Am J Physiol Renal Physiol (2005) 3.07
Ciliary dysfunction in developmental abnormalities and diseases. Curr Top Dev Biol (2008) 2.88
Dysfunctional cilia lead to altered ependyma and choroid plexus function, and result in the formation of hydrocephalus. Development (2005) 2.36
Mutant huntingtin directly increases susceptibility of mitochondria to the calcium-induced permeability transition and cytochrome c release. Hum Mol Genet (2004) 2.31
BMP canonical Smad signaling through Smad1 and Smad5 is required for endochondral bone formation. Development (2009) 2.21
Aberrant splicing of HTT generates the pathogenic exon 1 protein in Huntington disease. Proc Natl Acad Sci U S A (2013) 2.21
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
Primary cilia and signaling pathways in mammalian development, health and disease. Nephron Physiol (2009) 2.09
Cystin, a novel cilia-associated protein, is disrupted in the cpk mouse model of polycystic kidney disease. J Clin Invest (2002) 2.07
The power of automated high-resolution behavior analysis revealed by its application to mouse models of Huntington's and prion diseases. Proc Natl Acad Sci U S A (2007) 1.99
Early autophagic response in a novel knock-in model of Huntington disease. Hum Mol Genet (2010) 1.99
Conditional Kif3a ablation causes abnormal hedgehog signaling topography, growth plate dysfunction, and excessive bone and cartilage formation during mouse skeletogenesis. Development (2007) 1.97
The primary cilium in cell signaling and cancer. Cancer Res (2006) 1.97
Dramatic tissue-specific mutation length increases are an early molecular event in Huntington disease pathogenesis. Hum Mol Genet (2003) 1.96
Cilia proteins control cerebellar morphogenesis by promoting expansion of the granule progenitor pool. J Neurosci (2007) 1.92
Normal ciliogenesis requires synergy between the cystic kidney disease genes MKS-3 and NPHP-4. J Am Soc Nephrol (2010) 1.85
Wnt5a is required for proper mammary gland development and TGF-beta-mediated inhibition of ductal growth. Development (2007) 1.85
Sensory ciliogenesis in Caenorhabditis elegans: assignment of IFT components into distinct modules based on transport and phenotypic profiles. Mol Biol Cell (2007) 1.82
Heightened epithelial Na+ channel-mediated Na+ absorption in a murine polycystic kidney disease model epithelium lacking apical monocilia. Am J Physiol Cell Physiol (2005) 1.74
Loss of the Tg737 protein results in skeletal patterning defects. Dev Dyn (2003) 1.71
TGF-beta promotes Th17 cell development through inhibition of SOCS3. J Immunol (2009) 1.66
Mechanoregulation of intracellular Ca2+ concentration is attenuated in collecting duct of monocilium-impaired orpk mice. Am J Physiol Renal Physiol (2005) 1.66
Directional cell migration and chemotaxis in wound healing response to PDGF-AA are coordinated by the primary cilium in fibroblasts. Cell Physiol Biochem (2010) 1.63
DNA instability in postmitotic neurons. Proc Natl Acad Sci U S A (2008) 1.61
Functional redundancy of the B9 proteins and nephrocystins in Caenorhabditis elegans ciliogenesis. Mol Biol Cell (2008) 1.59
Rodent genetic models of Huntington disease. Neurobiol Dis (2008) 1.58
XBX-1 encodes a dynein light intermediate chain required for retrograde intraflagellar transport and cilia assembly in Caenorhabditis elegans. Mol Biol Cell (2003) 1.57
A novel dynein light intermediate chain colocalizes with the retrograde motor for intraflagellar transport at sites of axoneme assembly in chlamydomonas and Mammalian cells. Mol Biol Cell (2003) 1.56
A novel protein LZTFL1 regulates ciliary trafficking of the BBSome and Smoothened. PLoS Genet (2011) 1.49
Loss of primary cilia results in deregulated and unabated apical calcium entry in ARPKD collecting duct cells. Am J Physiol Renal Physiol (2006) 1.47
Transforming growth factor-beta signaling mediates hypoxia-induced pulmonary arterial remodeling and inhibition of alveolar development in newborn mouse lung. Am J Physiol Lung Cell Mol Physiol (2008) 1.45
The C. elegans homologs of nephrocystin-1 and nephrocystin-4 are cilia transition zone proteins involved in chemosensory perception. J Cell Sci (2005) 1.44
Development of the post-natal growth plate requires intraflagellar transport proteins. Dev Biol (2007) 1.44
TGF-β2 suppresses macrophage cytokine production and mucosal inflammatory responses in the developing intestine. Gastroenterology (2010) 1.44
Deletion of Tgfbr2 in Prx1-cre expressing mesenchyme results in defects in development of the long bones and joints. Dev Biol (2007) 1.42
Soluble levels of cytosolic tubulin regulate ciliary length control. Mol Biol Cell (2011) 1.41
Comparative gene expression profile analysis of GLI and c-MYC in an epithelial model of malignant transformation. Cancer Res (2002) 1.38
Disruption of IFT results in both exocrine and endocrine abnormalities in the pancreas of Tg737(orpk) mutant mice. Lab Invest (2005) 1.38
Identification of CHE-13, a novel intraflagellar transport protein required for cilia formation. Exp Cell Res (2003) 1.38
Longitudinal evaluation of the Hdh(CAG)150 knock-in murine model of Huntington's disease. J Neurosci (2007) 1.38
TGFbeta2 mediates the effects of hedgehog on hypertrophic differentiation and PTHrP expression. Development (2002) 1.37
The Oak Ridge Polycystic Kidney mouse: modeling ciliopathies of mice and men. Dev Dyn (2008) 1.36
Lack of primary cilia primes shear-induced endothelial-to-mesenchymal transition. Circ Res (2011) 1.31
Primary cilia are decreased in breast cancer: analysis of a collection of human breast cancer cell lines and tissues. J Histochem Cytochem (2010) 1.31
Primary cilia regulate Shh activity in the control of molar tooth number. Development (2009) 1.31
Loss of TGF-beta or Wnt5a results in an increase in Wnt/beta-catenin activity and redirects mammary tumour phenotype. Breast Cancer Res (2009) 1.29
A glucocorticoid-induced leucine-zipper protein, GILZ, inhibits adipogenesis of mesenchymal cells. EMBO Rep (2003) 1.27
Loss of apical monocilia on collecting duct principal cells impairs ATP secretion across the apical cell surface and ATP-dependent and flow-induced calcium signals. Purinergic Signal (2007) 1.27
Characterization of primary cilia and Hedgehog signaling during development of the human pancreas and in human pancreatic duct cancer cell lines. Dev Dyn (2008) 1.27
Dominant negative mutation of the TGF-beta receptor blocks hypoxia-induced pulmonary vascular remodeling. J Appl Physiol (1985) (2005) 1.25
IFTA-2 is a conserved cilia protein involved in pathways regulating longevity and dauer formation in Caenorhabditis elegans. J Cell Sci (2006) 1.24
An essential role for dermal primary cilia in hair follicle morphogenesis. J Invest Dermatol (2008) 1.22
Heat shock factor 1 regulates lifespan as distinct from disease onset in prion disease. Proc Natl Acad Sci U S A (2008) 1.20
Diminishing apoptosis by deletion of Bax or overexpression of Bcl-2 does not protect against infectious prion toxicity in vivo. J Neurosci (2007) 1.15
The zebrafish foxj1a transcription factor regulates cilia function in response to injury and epithelial stretch. Proc Natl Acad Sci U S A (2010) 1.15
Lymphotoxin-dependent prion replication in inflammatory stromal cells of granulomas. Immunity (2008) 1.14
The primary cilium coordinates early cardiogenesis and hedgehog signaling in cardiomyocyte differentiation. J Cell Sci (2009) 1.12
Cystic kidney diseases: all roads lead to the cilium. Physiology (Bethesda) (2004) 1.12
Mutations in Traf3ip1 reveal defects in ciliogenesis, embryonic development, and altered cell size regulation. Dev Biol (2011) 1.09
Caenorhabditis elegans DYF-2, an orthologue of human WDR19, is a component of the intraflagellar transport machinery in sensory cilia. Mol Biol Cell (2006) 1.08
Abnormal development of NG2+PDGFR-α+ neural progenitor cells leads to neonatal hydrocephalus in a ciliopathy mouse model. Nat Med (2012) 1.08
Disruption of PCP signaling causes limb morphogenesis and skeletal defects and may underlie Robinow syndrome and brachydactyly type B. Hum Mol Genet (2010) 1.07
Molecular profiling of the developing mouse axial skeleton: a role for Tgfbr2 in the development of the intervertebral disc. BMC Dev Biol (2010) 1.07
Cilia involvement in patterning and maintenance of the skeleton. Curr Top Dev Biol (2008) 1.06
Gene therapy rescues cilia defects and restores olfactory function in a mammalian ciliopathy model. Nat Med (2012) 1.05
In vivo evidence for NMDA receptor-mediated excitotoxicity in a murine genetic model of Huntington disease. J Neurosci (2009) 1.05
Role of epidermal primary cilia in the homeostasis of skin and hair follicles. Development (2011) 1.05
Tgfbr2 is required for development of the skull vault. Dev Biol (2009) 1.04
C-type natriuretic peptide regulates endochondral bone growth through p38 MAP kinase-dependent and -independent pathways. BMC Dev Biol (2007) 1.03
Prion pathogenesis is independent of caspase-12. Prion (2007) 1.03
WNT5A inhibits metastasis and alters splicing of Cd44 in breast cancer cells. PLoS One (2013) 1.02
Tgfbr2 regulates the maintenance of boundaries in the axial skeleton. Dev Biol (2006) 1.01
BBS mutations modify phenotypic expression of CEP290-related ciliopathies. Hum Mol Genet (2013) 0.99
Co-ordination of TGF-beta and FGF signaling pathways in bone organ cultures. Mech Dev (2004) 0.98
Leptin resistance is a secondary consequence of the obesity in ciliopathy mutant mice. Proc Natl Acad Sci U S A (2013) 0.98
Altered pH(i) regulation and Na(+)/HCO3(-) transporter activity in choroid plexus of cilia-defective Tg737(orpk) mutant mouse. Am J Physiol Cell Physiol (2006) 0.98
TGF-beta, c-Cbl, and PDGFR-alpha the in mammary stroma. Dev Biol (2005) 0.96
Proximal tubule proliferation is insufficient to induce rapid cyst formation after cilia disruption. J Am Soc Nephrol (2013) 0.96
PIKE-A is required for prolactin-mediated STAT5a activation in mammary gland development. EMBO J (2010) 0.96
Context dependent neuroprotective properties of prion protein (PrP). Prion (2009) 0.95
Chondrocyte-specific regulatory activity of Runx2 is essential for survival and skeletal development. Cells Tissues Organs (2011) 0.95
GMAP210 and IFT88 are present in the spermatid golgi apparatus and participate in the development of the acrosome-acroplaxome complex, head-tail coupling apparatus and tail. Dev Dyn (2011) 0.94
Runx2 regulates endochondral ossification through control of chondrocyte proliferation and differentiation. J Bone Miner Res (2014) 0.93
Nucleocytoplasmic transport signals affect the age at onset of abnormalities in knock-in mice expressing polyglutamine within an ectopic protein context. Hum Mol Genet (2003) 0.93
Mouse Huntington's disease homolog mRNA levels: variation and allele effects. Gene Expr (2004) 0.92