The primary cilium: a signalling centre during vertebrate development

No abstract is available for this paper.

• Publication year

  2010

• Venue

  Nature reviews genetics

• Publication date

  2010-05-01

• Fields of study

  Biology, Medicine

• Identifiers
  DOI 10.1038/nrg2774PMID 20395968PMCID PMC3121168
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

• I. INTRODUCTION

  2012cited by this paper
• Directional Cell Migration and Chemotaxis in Wound Healing Response to PDGF-AA are Coordinated by the Primary Cilium in Fibroblasts

  2010cited by this paper
• Independent regulation of Sox3 and Lmx1b by FGF and BMP signaling influences the neurogenic and non-neurogenic domains in the chick otic placode.

  2010cited by this paper
• FGF signalling during embryo development regulates cilia length in diverse epithelia

  2010cited by this paper
• Ofd1, a human disease gene, regulates the length and distal structure of centrioles.

  2010cited by this paper
• PCP effector gene Inturned is an important regulator of cilia formation and embryonic development in mammals.

  2010cited by this paper
• Loss of oriented cell division does not initiate cyst formation.

  2010cited by this paper
• The role of primary cilia in neuronal function.

  2010cited by this paper
• Bardet-Biedl Syndrome-associated Small GTPase ARL6 (BBS3) Functions at or near the Ciliary Gate and Modulates Wnt Signaling

  2010cited by this paper
• Hedgehog beyond medulloblastoma and basal cell carcinoma.

  2010cited by this paper
• Use of SNP array analysis to identify a novel TRIM32 mutation in limb-girdle muscular dystrophy type 2H.

  2009cited by this paper
• The primary cilium as a cellular signaling center: lessons from disease.

  2009cited by this paper
• Mouse Kif7/Costal2 is a cilia-associated protein that regulates Sonic hedgehog signaling

  2009cited by this paper
• Mutations in STIL, encoding a pericentriolar and centrosomal protein, cause primary microcephaly.

  2009cited by this paper
• Defective ciliogenesis, embryonic lethality and severe impairment of the Sonic Hedgehog pathway caused by inactivation of the mouse complex A intraflagellar transport gene Ift122/Wdr10, partially overlapping with the DNA repair gene Med1/Mbd4.

  2009cited by this paper
• Fused has evolved divergent roles in vertebrate Hedgehog signalling and motile ciliogenesis

  2009cited by this paper
• DYNC2H1 mutations cause asphyxiating thoracic dystrophy and short rib-polydactyly syndrome, type III.

  2009cited by this paper
• The Kinesin Protein Kif7 Is a Critical Regulator of Gli Transcription Factors in Mammalian Hedgehog Signaling

  2009cited by this paper
• Impaired Wnt–β-catenin signaling disrupts adult renal homeostasis and leads to cystic kidney ciliopathy

  2009cited by this paper
• The vertebrate primary cilium in development, homeostasis, and disease.

  2009cited by this paper
• Primary cilia can both mediate and suppress Hedgehog pathway–dependent tumorigenesis

  2009cited by this paper
• FGF Signaling during embryo development regulates cilia length in diverse epithelia

  2009cited by this paper
• Ultrastructural and antigenic properties of neural stem cells and their progeny in adult rat subventricular zone

  2009cited by this paper
• MKS3/TMEM67 mutations are a major cause of COACH Syndrome, a Joubert Syndrome related disorder with liver involvement

  2009cited by this paper
• The Talpid3 gene (KIAA0586) encodes a centrosomal protein that is essential for primary cilia formation

  2009cited by this paper
• Intraflagellar transport and the generation of dynamic, structurally and functionally diverse cilia.

  2009cited by this paper
• Planar cell polarity effector gene Fuzzy regulates cilia formation and Hedgehog signal transduction in mouse

  2009cited by this paper
• Advances in the pathogenesis and treatment of polycystic kidney disease

  2009cited by this paper
• The nonmotile ciliopathies

  2009cited by this paper
• Polycystic kidney disease.

  2009cited by this paper
• Localization of Inv in a distinctive intraciliary compartment requires the C-terminal ninein-homolog-containing region

  2009cited by this paper
• Cilium-independent regulation of Gli protein function by Sufu in Hedgehog signaling is evolutionarily conserved.

  2009cited by this paper
• Dampened Hedgehog signaling but normal Wnt signaling in zebrafish without cilia

  2009cited by this paper
• Polycystins and primary cilia: primers for cell cycle progression.

  2009cited by this paper
• Making sense of cilia in disease: The human ciliopathies

  2009cited by this paper
• Planarian Hh Signaling Regulates Regeneration Polarity and Links Hh Pathway Evolution to Cilia

  2009cited by this paper
• Suppressor of Fused inhibits mammalian Hedgehog signaling in the absence of cilia.

  2009cited by this paper
• A mouse model for Meckel syndrome reveals Mks1 is required for ciliogenesis and Hedgehog signaling.

  2009cited by this paper
• Dual and opposing roles of primary cilia in medulloblastoma development

  2009cited by this paper
• Paracrine Hedgehog signaling in cancer.

  2009cited by this paper
• The Zn finger protein Iguana impacts Hedgehog signaling by promoting ciliogenesis.

  2009cited by this paper
• Deficiency of the E3 ubiquitin ligase TRIM32 in mice leads to a myopathy with a neurogenic component.

  2009cited by this paper
• The planar cell polarity effector Fuz is essential for targeted membrane trafficking, ciliogenesis, and mouse embryonic development

  2009cited by this paper
• Lateral transport of Smoothened from the plasma membrane to the membrane of the cilium

  2009cited by this paper
• Fgf4 is required for left-right patterning of visceral organs in zebrafish.

  2009cited by this paper
• Cilia and the ciliopathies: An introduction

  2009cited by this paper
• The mammalian Cos2 homolog Kif7 plays an essential role in modulating Hh signal transduction during development.

  2009cited by this paper
• Mutations in the inositol polyphosphate-5-phosphatase E gene link phosphatidyl inositol signaling to the ciliopathies

  2009cited by this paper
• Primary Cilia Are Not Required for Normal Canonical Wnt Signaling in the Mouse Embryo

  2009cited by this paper
• Gli2 trafficking links Hedgehog-dependent activation of Smoothened in the primary cilium to transcriptional activation in the nucleus

  2009cited by this paper
• Ttc21b is required to restrict sonic hedgehog activity in the developing mouse forebrain.

  2009cited by this paper
• FGF-dependent left–right asymmetry patterning in zebrafish is mediated by Ier2 and Fibp1

  2009cited by this paper
• Retrograde Intraflagellar Transport Mutants Identify Complex A Proteins With Multiple Genetic Interactions in Chlamydomonas reinhardtii

  2009cited by this paper
• SHH Pathway and Cerebellar Development

  2009cited by this paper
• Dishevelled controls apical docking and planar polarization of basal bodies in ciliated epithelial cells

  2008cited by this paper
• Loss of nephrocystin-3 function can cause embryonic lethality, Meckel-Gruber-like syndrome, situs inversus, and renal-hepatic-pancreatic dysplasia.

  2008cited by this paper
• Costal2 functions as a kinesin-like protein in the hedgehog signal transduction pathway.

  2008cited by this paper
• THM1 negatively modulates mouse sonic hedgehog signal transduction and affects retrograde intraflagellar transport in cilia

  2008cited by this paper
• Mutations in the cilia gene ARL13B lead to the classical form of Joubert syndrome.

  2008cited by this paper
• Application of active and kinase-deficient kinome collection for identification of kinases regulating hedgehog signaling.

  2008cited by this paper
• The role of kinases in the Hedgehog signalling pathway

  2008cited by this paper
• Hedgehog signaling and primary cilia are required for the formation of adult neural stem cells

  2008cited by this paper
• Kif3a constrains β-catenin-dependent Wnt signalling through dual ciliary and non-ciliary mechanisms

  2008cited by this paper
• Loss of Fat4 disrupts PCP signaling and oriented cell division and leads to cystic kidney disease

  2008cited by this paper
• Bardet–Biedl syndrome proteins are required for the localization of G protein-coupled receptors to primary cilia

  2008cited by this paper
• Deletion of IFT20 in the mouse kidney causes misorientation of the mitotic spindle and cystic kidney disease

  2008cited by this paper
• Ciliary proteins link basal body polarization to planar cell polarity regulation

  2008cited by this paper
• elipsa is an early determinant of ciliogenesis that links the IFT particle to membrane-associated small GTPase Rab8

  2008cited by this paper
• Assembly of primary cilia

  2008cited by this paper
• Morphogenesis of the node and notochord: The cellular basis for the establishment and maintenance of left–right asymmetry in the mouse

  2008cited by this paper
• Intraflagellar transport, cilia, and mammalian Hedgehog signaling: Analysis in mouse embryonic fibroblasts

  2008cited by this paper
• Kinome siRNA Screen Identifies Regulators of Ciliogenesis and Hedgehog Signal Transduction

  2008cited by this paper
• The ciliary gene RPGRIP1L is mutated in cerebello-oculo-renal syndrome (Joubert syndrome type B) and Meckel syndrome

  2007cited by this paper
• Patched1 Regulates Hedgehog Signaling at the Primary Cilium

  2007cited by this paper
• Disruption of the basal body compromises proteasomal function and perturbs intracellular Wnt response

  2007cited by this paper
• Wnt5a functions in planar cell polarity regulation in mice.

  2007cited by this paper
• Wnt3 signaling in the epiblast is required for proper orientation of the anteroposterior axis.

  2007cited by this paper
• RhoA-mediated apical actin enrichment is required for ciliogenesis and promoted by Foxj1

  2007cited by this paper
• Mutations in the gene encoding the basal body protein RPGRIP1L, a nephrocystin-4 interactor, cause Joubert syndrome

  2007cited by this paper
• A role for the inositol kinase Ipk1 in ciliary beating and length maintenance

  2007cited by this paper
• Evc is a positive mediator of Ihh-regulated bone growth that localises at the base of chondrocyte cilia

  2007cited by this paper
• Wnt signaling in polycystic kidney disease.

  2007cited by this paper
• The graded response to Sonic Hedgehog depends on cilia architecture.

  2007cited by this paper
• Ftm is a novel basal body protein of cilia involved in Shh signalling

  2007cited by this paper
• IFT80, which encodes a conserved intraflagellar transport protein, is mutated in Jeune asphyxiating thoracic dystrophy

  2007cited by this paper
• Disruption of intraflagellar transport in adult mice leads to obesity and slow-onset cystic kidney disease.

  2007cited by this paper
• Identification of a novel BBS gene (BBS12) highlights the major role of a vertebrate-specific branch of chaperonin-related proteins in Bardet-Biedl syndrome.

  2007cited by this paper
• A core complex of BBS proteins cooperates with the GTPase Rab8 to promote ciliary membrane biogenesis.

  2007cited by this paper
• Cilia Proteins Control Cerebellar Morphogenesis by Promoting Expansion of the Granule Progenitor Pool

  2007cited by this paper
• The WD repeat-containing protein IFTA-1 is required for retrograde intraflagellar transport.

  2006cited by this paper
• Two separate molecular systems, Dachsous/Fat and Starry night/Frizzled, act independently to confer planar cell polarity

  2006cited by this paper
• The Role of Frizzled3 and Frizzled6 in Neural Tube Closure and in the Planar Polarity of Inner-Ear Sensory Hair Cells

  2006cited by this paper
• Asymmetric Localization of Vangl2 and Fz3 Indicate Novel Mechanisms for Planar Cell Polarity in Mammals

  2006cited by this paper
• GLI3-dependent transcriptional repression of Gli1, Gli2 and kidney patterning genes disrupts renal morphogenesis

  2006cited by this paper
• Control of Murine Kidney Development by Sonic Hedgehog and its GLI Effectors

  2006cited by this paper
• Defective planar cell polarity in polycystic kidney disease

  2006cited by this paper
• Ciliogenesis defects in embryos lacking inturned or fuzzy function are associated with failure of planar cell polarity and Hedgehog signaling

  2006cited by this paper
• BBS10 encodes a vertebrate-specific chaperonin-like protein and is a major BBS locus

  2006cited by this paper
• Oral-facial-digital type I protein is required for primary cilia formation and left-right axis specification

  2006cited by this paper
• Homozygosity mapping with SNP arrays identifies TRIM32, an E3 ubiquitin ligase, as a Bardet-Biedl syndrome gene (BBS11).

  2006cited by this paper

• The Role of Fibro/adipogenic Progenitors (FAPs) in Sarcopenia: Mechanisms and Potential Therapeutic Strategies

  2026cites this paper
• Orchestrating Microtubules: A Review of Kinase–Dependent Regulatory Mechanisms

  2026cites this paper
• Broadening horizons: new links between cilia and heart development and disease.

  2026cites this paper
• Disruption of the human cystin-1 myristoyl-electrostatic switch causes polycystic kidney disease that phenocopies autosomal recessive polycystic kidney disease.

  2026cites this paper
• TTBK2‐Driven Ciliogenesis Is Required for Intrinsic Neuronal Regeneration After Spinal Cord Injury

  2026cites this paper
• An ancient transcription factor functions as the master regulator of primary cilia formation

  2026cites this paper
• The Role of the Hedgehog Pathway in Alcohol-Induced Birth Defects.

  2026cites this paper
• Loss of SPECC1L in cranial neural crest cells results in increased hedgehog signaling and frontonasal dysplasia

  2026cites this paper
• Centrioles and Cellular Differentiation

  2026influential citation
• Unveiling a New Link: Cholesterol Deficiency in Smith-Lemli-Opitz and Niemann-Pick C as a Driver of Ciliopathies.

  2026cites this paper
• Machine learning based sensing of particle shape and size using passive artificial cilia

  2026cites this paper
• Three-dimensional approaches to measuring primary cilia in hippocampal neurons: A comparative analysis.

  2026cites this paper
• Emerging regulators of endothelial lipid metabolism in atherosclerosis.

  2026cites this paper
• Trisomy 21 alters ciliary localization of Sonic Hedgehog signaling proteins

  2026cites this paper
• Molecular Regulation of Palatogenesis and Clefting: An Integrative Analysis of Genetic, Epigenetic Networks, and Environmental Interactions

  2025cites this paper
• Smoothened inhibition of PKA at cilia transduces Hedgehog signals

  2025cites this paper
• Temporal ablation of the ciliary protein IFT88 alters normal brainwave patterns

  2025cites this paper
• Hedgehog pathway, cell cycle, and primary cilium

  2025cites this paper
• PCP protein Prickle 1 regulates Sertoli cell and testis function via cytoskeletal organization through the recruitment of multiple regulatory proteins.

  2025cites this paper
• Cleavage of the Meckel-Gruber syndrome protein TMEM67 by ADAMTS9 uncouples Wnt signaling and ciliogenesis

  2025cites this paper
• Identification of New Ciliary Signaling Pathways in the Brain and Insights into Neurological Disorders

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• A single-cell atlas of spatial and temporal gene expression in the mouse cranial neural plate

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• Alternative splicing categorizes organ development by stage and reveals unique human splicing variants linked to neuromuscular disorders

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• Dynein: A Multifaceted Therapeutic Target and Its Dysregulation in Aberrant Cell Proliferation.

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• The mechanism of FAPs in the regeneration of skeletal muscle

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• MUC5B Polymorphism and Susceptibility to Idiopathic Pulmonary Fibrosis in Morocco

  2025cites this paper
• Genetic Variants in KIF7 May Contribute to Supernumerary Tooth Formation

  2025cites this paper
• A differential requirement for ciliary transition zone proteins in human and mouse neural progenitor fate specification

  2025cites this paper
• HSD17B4 deficiency causes dysregulation of primary cilia and is alleviated by acetyl-CoA

  2025cites this paper
• The Mn-motif protein MAP6d1 assembles ciliary doublet microtubules

  2025cites this paper
• Collaborative role of two distinct cilium-specific cytoskeletal systems in driving Hedgehog-responsive transcription factor trafficking

  2025cites this paper
• Drug and siRNA screens identify ROCK2 as a therapeutic target for ciliopathies

  2025cites this paper
• Prominosomes - a particular class of extracellular vesicles containing prominin-1/CD133?

  2025cites this paper
• Receptor Allostery Promotes Context-Dependent Sonic Hedgehog Signaling During Embryonic Development

  2025cites this paper
• Proteomic profiling of primary cilia in the developing brain uncovers new regulators of cortical development

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• Primary cilia and cancer: a tale of many faces

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• A brief exposure to rotenone alters microtubule dynamics resulting in aberrant elongation of primary cilia with impaired function

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• GPC6 facilitates progression of SHH-subgroup medulloblastoma by enhancing Hedgehog secretion and signaling responses.

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• Renal ciliopathies.

  2025cites this paper
• Progressive tooth pattern changes in Cilk1-deficient mice depending on Hedgehog signaling

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• Primary cilia in cancer: structures, functions, mechanisms, and therapeutic implications

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• UNC119 regulates T-cell receptor signalling in primary T cells and T acute lymphocytic leukaemia

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• Ciliary and Non-Ciliary Roles of IFT88 in Development and Diseases

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• Dynamics of primary cilia in endothelial and mesenchymal cells throughout mouse lung development

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• Coordination of IFT20 With Other IFT Components Is Required for Ciliogenesis

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• Joubert syndrome 26 protein enforces compartmentalized motility of a ciliary kinesin.

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• The primary cilium as a gatekeeper of FGFR2 function.

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• Ophthalmologic Manifestations in Bardet–Biedl Syndrome: Emerging Therapeutic Approaches

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• Knocking out ARL13B completely abolishes primary ciliogenesis in cell lines

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• Multicilia dynamically transduce Sonic Hedgehog signaling to regulate choroid plexus functions.

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• Primary ciliary protein kinase A activity in the prefrontal cortex modulates stress in mice.

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• Whole-Mount Visualization of Primary Cilia in the Developing Mouse Brain

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• The primary cilium as a multifunctional organelle: emerging roles and unanswered questions

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• Tackling ciliary specialization to understand phenotypic variability in human primary ciliopathies

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• Primary cilia in neural development and disease.

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• Primary Cilia‐Mediated Hedgehog Signaling Regulates Cell Fate During ER Stress‐Induced Life or Death Decisions

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• Taking Down the Primary Cilium: Pathways for Disassembly in Differentiating Cells

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• The kinesin-4 protein KIF27 forms a cytoskeletal scaffold at the transition zone to promote motile cilia structural integrity.

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• Emerging insights into primary ciliary dyskinesia-associated hydrocephalus: a scoping review

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• Different temporal dynamics of primary cilia formation and elongation during adipocyte differentiation in umbilical cord- and bone marrow-derived mesenchymal stem cells.

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• TMEM216 promotes primary ciliogenesis and Hedgehog signaling through the SUFU-GLI2/GLI3 axis

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