The Graded Response to Sonic Hedgehog Depends on Cilia Architecture

Altered Neural Cell Fates and Medulloblastoma in Mouse patched Mutants

Goodrich, L. V., Milenković, L., Higgins, K. M., & Scott, M. P. (1997) · Science · doi:10.1126/science.277.5329.1109 · Cited by 1,696

Vertebrate Smoothened functions at the primary cilium

Corbit, K. C., Aanstad, P., Singla, V., Norman, A. R., Stainier, D. Y. R., & Reiter, J. F. (2005) · Nature · doi:10.1038/nature04117 · Cited by 1,481

Hedgehog-Regulated Processing of Gli3 Produces an Anterior/Posterior Repressor Gradient in the Developing Vertebrate Limb

Wang, B., Fallon, J. F., & Beachy, P. A. (2000) · Cell · doi:10.1016/s0092-8674(00)80678-9 · Cited by 1,014

Gli2 and Gli3 Localize to Cilia and Require the Intraflagellar Transport Protein Polaris for Processing and Function

Haycraft, C. J., Banizs, B., Aydin-Son, Y., Zhang, Q., Michaud, E. J., & Yoder, B. K. (2005) · PLoS Genetics · doi:10.1371/journal.pgen.0010053 · Cited by 902

Cilia and Hedgehog responsiveness in the mouse

Huangfu, D., & Anderson, K. V. (2005) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0505328102 · Cited by 820

Expression of primary cilia in mammalian cells

WHEATLEY, D. N., WANG, A. M., & STRUGNELL, G. E. (1996) · Cell Biology International · doi:10.1006/cbir.1996.0011 · Cited by 378

Hippi is essential for node cilia assembly and Sonic hedgehog signaling

Houde, C., Dickinson, R. J., Houtzager, V. M., Cullum, R., Montpetit, R., Metzler, M., Simpson, E. M., Roy, S., Hayden, M. R., Hoodless, P. A., et al. (2006) · Developmental Biology · doi:10.1016/j.ydbio.2006.09.001 · Cited by 98

Ror2 signaling regulates Golgi structure and transport through IFT20 for tumor invasiveness

Nishita, M., Park, S.-Y., Nishio, T., Kamizaki, K., Wang, Z., Tamada, K., Takumi, T., Hashimoto, R., Otani, H., Pazour, G. J., et al. (2017) · Scientific Reports · doi:10.1038/s41598-016-0028-x · Cited by 15,947

The primary cilium: a signalling centre during vertebrate development

Goetz, S. C., & Anderson, K. V. (2010) · Nature Reviews Genetics · doi:10.1038/nrg2774 · Cited by 1,824

Hedgehog: functions and mechanisms

Varjosalo, M., & Taipale, J. (2008) · Genes & Development · doi:10.1101/gad.1693608 · Cited by 1,217

Primary Cilia and Mammalian Hedgehog Signaling

Bangs, F., & Anderson, K. V. (2017) · Cold Spring Harbor Perspectives in Biology · doi:10.1101/cshperspect.a028175 · Cited by 642

The Ciliary G-Protein-Coupled Receptor Gpr161 Negatively Regulates the Sonic Hedgehog Pathway via cAMP Signaling

Mukhopadhyay, S., Wen, X., Ratti, N., Loktev, A., Rangell, L., Scales, S. J., & Jackson, P. K. (2013) · Cell · doi:10.1016/j.cell.2012.12.026 · Cited by 486

Primary cilia as dynamic and diverse signalling hubs in development and disease

Mill, P., Christensen, S. T., & Pedersen, L. B. (2023) · Nature Reviews Genetics · doi:10.1038/s41576-023-00587-9 · Cited by 417

Mutations in INPP5E, encoding inositol polyphosphate-5-phosphatase E, link phosphatidyl inositol signaling to the ciliopathies

Bielas, S. L., Silhavy, J. L., Brancati, F., Kisseleva, M. V., Al-Gazali, L., Sztriha, L., Bayoumi, R. A., Zaki, M. S., Abdel-Aleem, A., Rosti, R. O., et al. (2009) · Nature Genetics · doi:10.1038/ng.423 · Cited by 396

Functional dissection of Rab GTPases involved in primary cilium formation

Yoshimura, S.-I., Egerer, J., Fuchs, E., Haas, A. K., & Barr, F. A. (2007) · The Journal of Cell Biology · doi:10.1083/jcb.200703047 · Cited by 375

Phosphoinositides Regulate Ciliary Protein Trafficking to Modulate Hedgehog Signaling

Garcia-Gonzalo, F. R., Phua, S. C., Roberson, E. C., Garcia, G., Abedin, M., Schurmans, S., Inoue, T., & Reiter, J. F. (2015) · Developmental Cell · doi:10.1016/j.devcel.2015.08.001 · Cited by 330

THM1 negatively modulates mouse sonic hedgehog signal transduction and affects retrograde intraflagellar transport in cilia

Tran, P. V., Haycraft, C. J., Besschetnova, T. Y., Turbe-Doan, A., Stottmann, R. W., Herron, B. J., Chesebro, A. L., Qiu, H., Scherz, P. J., Shah, J. V., et al. (2008) · Nature Genetics · doi:10.1038/ng.105 · Cited by 322

The kinesin-4 protein Kif7 regulates mammalian Hedgehog signalling by organizing the cilium tip compartment

He, M., Subramanian, R., Bangs, F., Omelchenko, T., Liem Jr, K. F., Kapoor, T. M., & Anderson, K. V. (2014) · Nature Cell Biology · doi:10.1038/ncb2988 · Cited by 302

Primary Cilia in the Developing and Mature Brain

Guemez-Gamboa, A., Coufal, N. G., & Gleeson, J. G. (2014) · Neuron · doi:10.1016/j.neuron.2014.04.024 · Cited by 296

Mouse Kif7/Costal2 is a cilia-associated protein that regulates Sonic hedgehog signaling

Liem, K. F., He, M., Ocbina, P. J. R., & Anderson, K. V. (2009) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0906944106 · Cited by 300

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

Dhekne, H. S., Yanatori, I., Gomez, R. C., Tonelli, F., Diez, F., Schüle, B., Steger, M., Alessi, D. R., & Pfeffer, S. R. (2018) · eLife · doi:10.7554/elife.40202 · Cited by 240

Subcellular spatial regulation of canonical Wnt signalling at the primary cilium

Lancaster, M. A., Schroth, J., & Gleeson, J. G. (2011) · Nature Cell Biology · doi:10.1038/ncb2259 · Cited by 267

Primary cilia in neurodevelopmental disorders

Valente, E. M., Rosti, R. O., Gibbs, E., & Gleeson, J. G. (2014) · Nature Reviews Neurology · doi:10.1038/nrneurol.2013.247 · Cited by 254

Dampened Hedgehog signaling but normal Wnt signaling in zebrafish without cilia

Huang, P., & Schier, A. F. (2009) · Development · doi:10.1242/dev.041343 · Cited by 210

The IFT-A complex regulates Shh signaling through cilia structure and membrane protein trafficking

Liem, K. F., Ashe, A., He, M., Satir, P., Moran, J., Beier, D., Wicking, C., & Anderson, K. V. (2012) · Journal of Cell Biology · doi:10.1083/jcb.201110049 · Cited by 214

Cilia in the nervous system: linking cilia function and neurodevelopmental disorders

Lee, J. E., & Gleeson, J. G. (2011) · Current Opinion in Neurology · doi:10.1097/wco.0b013e3283444d05 · Cited by 185

Intraflagellar transport protein 122 antagonizes Sonic Hedgehog signaling and controls ciliary localization of pathway components

Qin, J., Lin, Y., Norman, R. X., Ko, H. W., & Eggenschwiler, J. T. (2011) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.1011410108 · Cited by 176

Apical Abscission Alters Cell Polarity and Dismantles the Primary Cilium During Neurogenesis

Das, R. M., & Storey, K. G. (2014) · Science · doi:10.1126/science.1247521 · Cited by 182

Lineage specificity of primary cilia in the mouse embryo

Bangs, F. K., Schrode, N., Hadjantonakis, A.-K., & Anderson, K. V. (2015) · Nature Cell Biology · doi:10.1038/ncb3091 · Cited by 174

A Smoothened-Evc2 Complex Transduces the Hedgehog Signal at Primary Cilia

Dorn, K. V., Hughes, C. E., & Rohatgi, R. (2012) · Developmental Cell · doi:10.1016/j.devcel.2012.07.004 · Cited by 174

The role of primary cilia in the development and disease of the retina

Wheway, G., Parry, D. A., & Johnson, C. A. (2014) · Organogenesis · doi:10.4161/org.26710 · Cited by 152

Primary Cilia Signaling Shapes the Development of Interneuronal Connectivity

Guo, J., Otis, J. M., Higginbotham, H., Monckton, C., Cheng, J., Asokan, A., Mykytyn, K., Caspary, T., Stuber, G. D., & Anton, E. S. (2017) · Developmental Cell · doi:10.1016/j.devcel.2017.07.010 · Cited by 136

Tubby-like protein 3 (TULP3) regulates patterning in the mouse embryo through inhibition of Hedgehog signaling

Norman, R. X., Ko, H. W., Huang, V., Eun, C. M., Abler, L. L., Zhang, Z., Sun, X., & Eggenschwiler, J. T. (2009) · Human Molecular Genetics · doi:10.1093/hmg/ddp113 · Cited by 103

RAB8, RAB10 and RILPL1 contribute to both LRRK2 kinase–mediated centrosomal cohesion and ciliogenesis deficits

Lara Ordónez, A. J., Fernández, B., Fdez, E., Romo-Lozano, M., Madero-Pérez, J., Lobbestael, E., Baekelandt, V., Aiastui, A., López de Munaín, A., Melrose, H. L., et al. (2019) · Human Molecular Genetics · doi:10.1093/hmg/ddz201 · Cited by 103

Mouse hitchhiker mutants have spina bifida, dorso-ventral patterning defects and polydactyly: identification of Tulp3 as a novel negative regulator of the Sonic hedgehog pathway

Patterson, V. L., Damrau, C., Paudyal, A., Reeve, B., Grimes, D. T., Stewart, M. E., Williams, D. J., Siggers, P., Greenfield, A., & Murdoch, J. N. (2009) · Human Molecular Genetics · doi:10.1093/hmg/ddp075 · Cited by 101

Primary Cilia Signaling Promotes Axonal Tract Development and Is Disrupted in Joubert Syndrome-Related Disorders Models

Guo, J., Otis, J. M., Suciu, S. K., Catalano, C., Xing, L., Constable, S., Wachten, D., Gupton, S., Lee, J., Lee, A., et al. (2019) · Developmental Cell · doi:10.1016/j.devcel.2019.11.005 · Cited by 100

A Cell/Cilia Cycle Biosensor for Single-Cell Kinetics Reveals Persistence of Cilia after G1/S Transition Is a General Property in Cells and Mice

Ford, M. J., Yeyati, P. L., Mali, G. R., Keighren, M. A., Waddell, S. H., Mjoseng, H. K., Douglas, A. T., Hall, E. A., Sakaue-Sawano, A., Miyawaki, A., et al. (2018) · Developmental Cell · doi:10.1016/j.devcel.2018.10.027 · Cited by 89

Cilia and coordination of signaling networks during heart development

Koefoed, K., Veland, I. R., Pedersen, L. B., Larsen, L. A., & Christensen, S. T. (2014) · Organogenesis · doi:10.4161/org.27483 · Cited by 84

Dysfunction of the ciliary ARMC9/TOGARAM1 protein module causes Joubert syndrome

Latour, B. L., Van De Weghe, J. C., Rusterholz, T. D. S., Letteboer, S. J. F., Gomez, A., Shaheen, R., Gesemann, M., Karamzade, A., Asadollahi, M., Barroso-Gil, M., et al. (2020) · Journal of Clinical Investigation · doi:10.1172/jci131656 · Cited by 76

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

Tereshko, L., Gao, Y., Cary, B. A., Turrigiano, G. G., & Sengupta, P. (2021) · eLife · doi:10.7554/elife.65427 · Cited by 60

Quantitative comparison of primary cilia marker expression and length in the mouse brain

Sipos, É., Komoly, S., & Ács, P. (2018) · Journal of Molecular Neuroscience · doi:10.1007/s12031-018-1036-z · Cited by 61

Ranbp1 , Deleted in DiGeorge/22q11.2 Deletion Syndrome, is a Microcephaly Gene That Selectively Disrupts Layer 2/3 Cortical Projection Neuron Generation

Paronett, E. M., Meechan, D. W., Karpinski, B. A., LaMantia, A.-S., & Maynard, T. M. (2015) · Cerebral Cortex · doi:10.1093/cercor/bhu285 · Cited by 54

Lack of Dopaminergic Inputs Elongates the Primary Cilia of Striatal Neurons

Miyoshi, K., Kasahara, K., Murakami, S., Takeshima, M., Kumamoto, N., Sato, A., Miyazaki, I., Matsuzaki, S., Sasaoka, T., Katayama, T., et al. (2014) · PLoS ONE · doi:10.1371/journal.pone.0097918 · Cited by 51

Functional characterization of tektin-1 in motile cilia and evidence for TEKT1 as a new candidate gene for motile ciliopathies

Ryan, R., Failler, M., Reilly, M. L., Garfa-Traore, M., Delous, M., Filhol, E., Reboul, T., Bole-Feysot, C., Nitschké, P., Baudouin, V., et al. (2018) · Human Molecular Genetics · doi:10.1093/hmg/ddx396 · Cited by 48

Primary cilia TRP channel regulates hippocampal excitability

Vien, T. N., Ta, M. C., Kimura, L. F., Onay, T., & DeCaen, P. G. (2023) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.2219686120 · Cited by 33

Interleukin-1β sequesters hypoxia inducible factor 2α to the primary cilium

Wann, A. K., Thompson, C. L., Chapple, J. P., & Knight, M. M. (2013) · Cilia · doi:10.1186/2046-2530-2-17 · Cited by 38

Primary cilia signaling in astrocytes mediates development and regional-specific functional specification

Wang, L., Guo, Q., Acharya, S., Zheng, X., Huynh, V., Whitmore, B., Yimit, A., Malhotra, M., Chatterji, S., Rosin, N., et al. (2024) · Nature Neuroscience · doi:10.1038/s41593-024-01726-z · Cited by 33

Endocytic recycling protein EHD1 regulates primary cilia morphogenesis and SHH signaling during neural tube development

Bhattacharyya, S., Rainey, M. A., Arya, P., Mohapatra, B. C., Mushtaq, I., Dutta, S., George, M., Storck, M. D., McComb, R. D., Muirhead, D., et al. (2016) · Scientific Reports · doi:10.1038/srep20727 · Cited by 36

G‐protein‐coupled receptors and localized signaling in the primary cilium during ventral neural tube patterning

Hwang, S., & Mukhopadhyay, S. (2015) · Birth Defects Research Part A: Clinical and Molecular Teratology · doi:10.1002/bdra.23267 · Cited by 20

Primary cilia influence progenitor function during cortical development

Zaidi, D., Chinnappa, K., & Francis, F. (2022) · Cells · doi:10.3390/cells11182895 · Cited by 19

MAP9/MAPH-9 supports axonemal microtubule doublets and modulates motor movement

Tran, M. V., Khuntsariya, D., Fetter, R. D., Ferguson, J. W., Wang, J. T., Long, A. F., Cote, L. E., Wellard, S. R., Vázquez-Martínez, N., Sallee, M. D., et al. (2024) · Developmental Cell · doi:10.1016/j.devcel.2023.12.001 · Cited by 18

Cilia in cell signaling and human disorders

Duldulao, N. A., Li, J., & Sun, Z. (2010) · Protein & Cell · doi:10.1007/s13238-010-0098-7 · Cited by 13

Tau-tubulin kinase 2 restrains microtubule-depolymerizer KIF2A to support primary cilia growth

Benk Vysloužil, D., Bernatík, O., Lánská, E., Renzová, T., Binó, L., Lacigová, A., Drahošová, T., Lánský, Z., & Čajánek, L. (2025) · Cell Communication and Signaling · doi:10.1186/s12964-025-02072-8 · Cited by 11

Hedgehog signalling is involved in acquired resistance to KRASG12C inhibitors in lung cancer cells

Lee, C., Yi, J., Park, J., Ahn, B., Won, Y.-W., Jeon, J., Lee, B. J., Cho, W. J., & Park, J. W. (2024) · Cell Death & Disease · doi:10.1038/s41419-024-06436-9 · Cited by 9