{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,5]],"date-time":"2026-09-05T14:10:26Z","timestamp":1788617426035,"version":"build-2803163510"},"reference-count":134,"publisher":"Pharmaceutical Society of Japan","issue":"9","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Biological & Pharmaceutical Bulletin","Biol. Pharm. Bull."],"published-print":{"date-parts":[[2026,9,1]]},"DOI":"10.1248\/bpb.b26-00284","type":"journal-article","created":{"date-parts":[[2026,8,31]],"date-time":"2026-08-31T22:18:31Z","timestamp":1788214711000},"page":"1382-1394","source":"Crossref","is-referenced-by-count":1,"title":["Ependymal Cells and Hydrocephalus: Recent Advances in Molecular Mechanisms"],"prefix":"10.1248","volume":"49","author":[{"given":"Shinya","family":"Ohata","sequence":"first","affiliation":[{"name":"Research Institute of Pharmaceutical Sciences, Faculty of Pharmacy, Musashino University"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maki","family":"Takagishi","sequence":"additional","affiliation":[{"name":"Department of Biomedical Science, Graduate School of Pharmaceutical Sciences, Nagoya City University"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"163","reference":[{"key":"1","doi-asserted-by":"crossref","unstructured":"1) Kelley DH, Thomas JH. Cerebrospinal fluid flow. Annu. Rev. Fluid Mech.<\/i>, 55<\/b>, 237\u2013264 (2023).","DOI":"10.1146\/annurev-fluid-120720-011638"},{"key":"2","doi-asserted-by":"crossref","unstructured":"2) Ohata S, Alvarez-Buylla A. Planar organization of multiciliated ependymal (E1) cells in the brain ventricular epithelium. Trends Neurosci.<\/i>, 39<\/b>, 543\u2013551 (2016).","DOI":"10.1016\/j.tins.2016.05.004"},{"key":"3","doi-asserted-by":"crossref","unstructured":"3) Ji W, Tang Z, Chen Y, Wang C, Tan C, Liao J, Tong L, Xiao G. Ependymal cilia: physiology and role in hydrocephalus. Front. Mol. Neurosci.<\/i>, 15<\/b>, 927479 (2022).","DOI":"10.3389\/fnmol.2022.927479"},{"key":"4","doi-asserted-by":"crossref","unstructured":"4) Del Bigio MR. History of research concerning the ependyma: a view from inside the human brain. Front. Cell. Neurosci.<\/i>, 17<\/b>, 1320369 (2024).","DOI":"10.3389\/fncel.2023.1320369"},{"key":"5","doi-asserted-by":"crossref","unstructured":"5) Groh AMR, Song YL, Tea F, Lu B, Huynh S, Afanasiev E, Bigotte M, Del Bigio MR, Stratton JA. Multiciliated ependymal cells: an update on biology and pathology in the adult brain. Acta Neuropathol.<\/i>, 148<\/b>, 39 (2024).","DOI":"10.1007\/s00401-024-02826-7"},{"key":"6","doi-asserted-by":"crossref","unstructured":"6) Groh AMR, Hodgson L, Bzdok D, Stratton JA. Follow the CSF flow: probing multiciliated ependymal cells in brain pathology. Trends Mol. Med.<\/i>, 31<\/b>, 409\u2013412 (2025).","DOI":"10.1016\/j.molmed.2024.10.007"},{"key":"7","doi-asserted-by":"crossref","unstructured":"7) Mirzadeh Z, Merkle FT, Soriano-Navarro M, Garcia-Verdugo JM, Alvarez-Buylla A. Neural stem cells confer unique pinwheel architecture to the ventricular surface in neurogenic regions of the adult brain. Cell Stem Cell<\/i>, 3<\/b>, 265\u2013278 (2008).","DOI":"10.1016\/j.stem.2008.07.004"},{"key":"8","doi-asserted-by":"crossref","unstructured":"8) Mirzadeh Z, Kusne Y, Duran-Moreno M, Cabrales E, Gil-Perotin S, Ortiz C, Chen B, Garcia-Verdugo JM, Sanai N, Alvarez-Buylla A. Bi- and uniciliated ependymal cells define continuous floor-plate-derived tanycytic territories. Nat. Commun.<\/i>, 8<\/b>, 13759 (2017).","DOI":"10.1038\/ncomms13759"},{"key":"9","doi-asserted-by":"crossref","unstructured":"9) Dali R, Estrada-Meza J, Langlet F. Tanycyte, the neuron whisperer. Physiol. Behav.<\/i>, 263<\/b>, 114108 (2023).","DOI":"10.1016\/j.physbeh.2023.114108"},{"key":"10","doi-asserted-by":"crossref","unstructured":"10) Bramall AN, Anton ES, Kahle KT, Fecci PE. Navigating the ventricles: Novel insights into the pathogenesis of hydrocephalus. EBioMedicine<\/i>, 78<\/b>, 103931 (2022).","DOI":"10.1016\/j.ebiom.2022.103931"},{"key":"11","doi-asserted-by":"crossref","unstructured":"11) Anwar F, Zhang K, Sun C, Pang M, Zhou W, Li H, He R, Liu X, Ming D. Hydrocephalus: An update on latest progress in pathophysiological and therapeutic research. Biomed. Pharmacother.<\/i>, 181<\/b>, 117702 (2024).","DOI":"10.1016\/j.biopha.2024.117702"},{"key":"12","doi-asserted-by":"crossref","unstructured":"12) Duy PQ, Greenberg ABW, Butler WE, Kahle KT. Rethinking the cilia hypothesis of hydrocephalus. Neurobiol. Dis.<\/i>, 175<\/b>, 105913 (2022).","DOI":"10.1016\/j.nbd.2022.105913"},{"key":"13","doi-asserted-by":"crossref","unstructured":"13) Bigotte M, Groh AMR, Afanasiev E, et al.<\/i> Cerebrospinal fluid-driven ependymal motile cilia defects are implicated in multiple sclerosis. Brain<\/i>, awaf440 (2025).","DOI":"10.1101\/2025.07.06.663378"},{"key":"14","doi-asserted-by":"crossref","unstructured":"14) Xie H, Kang Y, Liu J, Huang M, Dai Z, Shi J, Wang S, Li L, Li Y, Zheng P, Sun Y, Han Q, Zhang J, Zhu Z, Xu L, Yelick PC, Cao M, Zhao C. Ependymal polarity defects coupled with disorganized ciliary beating drive abnormal cerebrospinal fluid flow and spine curvature in zebrafish. PLoS Biol.<\/i>, 21<\/b>, e3002008 (2023).","DOI":"10.1371\/journal.pbio.3002008"},{"key":"15","doi-asserted-by":"crossref","unstructured":"15) Spassky N, Meunier A. The development and functions of multiciliated epithelia. Nat. Rev. Mol. Cell Biol.<\/i>, 18<\/b>, 423\u2013436 (2017).","DOI":"10.1038\/nrm.2017.21"},{"key":"16","doi-asserted-by":"crossref","unstructured":"16) O\u2019Callaghan C, Sikand K, Rutman A. Respiratory and brain ependymal ciliary function. Pediatr. Res.<\/i>, 46<\/b>, 704\u2013707 (1999).","DOI":"10.1203\/00006450-199912000-00005"},{"key":"17","doi-asserted-by":"crossref","unstructured":"17) O\u2019Callaghan C, Sikand K, Chilvers MA. Analysis of ependymal ciliary beat pattern and beat frequency using high speed imaging: comparison with the photomultiplier and photodiode methods. Cilia<\/i>, 1<\/b>, 8 (2012).","DOI":"10.1186\/2046-2530-1-8"},{"key":"18","doi-asserted-by":"crossref","unstructured":"18) Omran AJA, Saternos HC, Althobaiti YS, Wisner A, Sari Y, Nauli SM, AbouAlaiwi WA. Alcohol consumption impairs the ependymal cilia motility in the brain ventricles. Sci. Rep.<\/i>, 7<\/b>, 13652 (2017).","DOI":"10.1038\/s41598-017-13947-3"},{"key":"19","doi-asserted-by":"crossref","unstructured":"19) Hilgendorf KI, Myers BR, Reiter JF. Emerging mechanistic understanding of cilia function in cellular signalling. Nat. Rev. Mol. Cell Biol.<\/i>, 25<\/b>, 555\u2013573 (2024).","DOI":"10.1038\/s41580-023-00698-5"},{"key":"20","doi-asserted-by":"crossref","unstructured":"20) Walton T, Gui M, Velkova S, Fassad MR, Hirst RA, Haarman E, O\u2019Callaghan C, Bottier M, Burgoyne T, Mitchison HM, Brown A. Axonemal structures reveal mechanoregulatory and disease mechanisms. Nature<\/i>, 618<\/b>, 625\u2013633 (2023).","DOI":"10.1038\/s41586-023-06140-2"},{"key":"21","doi-asserted-by":"crossref","unstructured":"21) Lin J, Nicastro D. Asymmetric distribution and spatial switching of dynein activity generates ciliary motility. Science<\/i>, 360<\/b>, eaar1968 (2018).","DOI":"10.1126\/science.aar1968"},{"key":"22","doi-asserted-by":"crossref","unstructured":"22) Kunimoto K, Yamazaki Y, Nishida T, Shinohara K, Ishikawa H, Hasegawa T, Okanoue T, Hamada H, Noda T, Tamura A, Tsukita S, Tsukita S. Coordinated ciliary beating requires Odf2-mediated polarization of basal bodies via<\/i> basal feet. Cell<\/i>, 148<\/b>, 189\u2013200 (2012).","DOI":"10.1016\/j.cell.2011.10.052"},{"key":"23","doi-asserted-by":"crossref","unstructured":"23) Nguyen QPH, Liu Z, Albulescu A, Ouyang H, Zlock L, Coyaud E, Laurent E, Finkbeiner W, Moraes TJ, Raught B, Mennella V. Comparative super-resolution mapping of basal feet reveals a modular but distinct architecture in primary and motile cilia. Dev. Cell<\/i>, 55<\/b>, 209\u2013223.e7 (2020).","DOI":"10.1016\/j.devcel.2020.09.015"},{"key":"24","doi-asserted-by":"crossref","unstructured":"24) Lee H, Lee J, Shin M, Goutam RS, Kim J, Park S. DEUP1 functions as a scaffold for basal foot integrity and planar polarity in multiciliated cells. Nat. Commun.<\/i>, 17<\/b>, 3875 (2026).","DOI":"10.1038\/s41467-026-70661-3"},{"key":"25","doi-asserted-by":"crossref","unstructured":"25) Yang J, Liu X, Yue G, Adamian M, Bulgakov O, Li T. Rootletin, a novel coiled-coil protein, is a structural component of the ciliary rootlet. J. Cell Biol.<\/i>, 159<\/b>, 431\u2013440 (2002).","DOI":"10.1083\/jcb.200207153"},{"key":"26","doi-asserted-by":"crossref","unstructured":"26) Ko D, Kim J, Rhee K, Choi H-J. Identification of a structurally dynamic domain for oligomer formation in rootletin. J. Mol. Biol.<\/i>, 432<\/b>, 3915\u20133932 (2020).","DOI":"10.1016\/j.jmb.2020.04.012"},{"key":"27","doi-asserted-by":"crossref","unstructured":"27) Ringers C, Bialonski S, Ege M, Solovev A, Hansen JN, Jeong I, Friedrich BM, Jurisch-Yaksi N. Novel analytical tools reveal that local synchronization of cilia coincides with tissue-scale metachronal waves in zebrafish multiciliated epithelia. eLife<\/i>, 12<\/b>, e77701 (2023).","DOI":"10.7554\/eLife.77701"},{"key":"28","doi-asserted-by":"crossref","unstructured":"28) Werner ME, Hwang P, Huisman F, Taborek P, Yu CC, Mitchell BJ. Actin and microtubules drive differential aspects of planar cell polarity in multiciliated cells. J. Cell Biol.<\/i>, 195<\/b>, 19\u201326 (2011).","DOI":"10.1083\/jcb.201106110"},{"key":"29","doi-asserted-by":"crossref","unstructured":"29) Mahuzier A, Shihavuddin A, Fournier C, Lansade P, Faucourt M, Menezes N, Meunier A, Garfa-Traor\u00e9 M, Carlier M-F, Voituriez R, Genovesio A, Spassky N, Delgehyr N. Ependymal cilia beating induces an actin network to protect centrioles against shear stress. Nat. Commun.<\/i>, 9<\/b>, 2279 (2018).","DOI":"10.1038\/s41467-018-04676-w"},{"key":"30","doi-asserted-by":"crossref","unstructured":"30) Nguyen T, Chin W, O\u2019Brien JA, Verdugo P, Berger AJ. Intracellular pathways regulating ciliary beating of rat brain ependymal cells. J. Physiol.<\/i>, 531<\/b>, 131\u2013140 (2001).","DOI":"10.1111\/j.1469-7793.2001.0131j.x"},{"key":"31","doi-asserted-by":"crossref","unstructured":"31) Genzen JR, Yang D, Ravid K, Bordey A. Activation of adenosine A2B receptors enhances ciliary beat frequency in mouse lateral ventricle ependymal cells. Cerebrospinal Fluid Res.<\/i>, 6<\/b>, 15 (2009).","DOI":"10.1186\/1743-8454-6-15"},{"key":"32","doi-asserted-by":"crossref","unstructured":"32) Kawaguchi K, Tsuji S, Hirao T, Liu Y, Boshi Z, Asano S. Adenosine stimulates beating of neonatal brain-derived cilia through adenosine A2B<\/sub> receptor on the cilia and activation of protein kinase A pathway. Biol. Pharm. Bull.<\/i>, 47<\/b>, 1113\u20131118 (2024).","DOI":"10.1248\/bpb.b23-00913"},{"key":"33","doi-asserted-by":"crossref","unstructured":"33) Conductier G, Brau F, Viola A, Langlet F, Ramkumar N, Dehouck B, Lemaire T, Chapot R, Lucas L, Rov\u00e8re C, Maitre P, Hosseiny S, Petit-Paitel A, Adamantidis A, Lakaye B, Risold P-Y, Pr\u00e9vot V, Meste O, Nahon J-L, Guyon A. Melanin-concentrating hormone regulates beat frequency of ependymal cilia and ventricular volume. Nat. Neurosci.<\/i>, 16<\/b>, 845\u2013847 (2013).","DOI":"10.1038\/nn.3401"},{"key":"34","unstructured":"34) Merveille A-C, Davis EE, Becker-Heck A, et al.<\/i> CCDC39 is required for assembly of inner dynein arms and the dynein regulatory complex and for normal ciliary motility in humans and dogs. Nat. Genet.<\/i>, 43<\/b>, 72\u201378 (2011)."},{"key":"35","doi-asserted-by":"crossref","unstructured":"35) Antony D, Becker-Heck A, Zariwala MA, et al.<\/i> Mutations in CCDC39<\/i> and CCDC40<\/i> are the major cause of primary ciliary dyskinesia with axonemal disorganization and absent inner dynein arms. Hum. Mutat.<\/i>, 34<\/b>, 462\u2013472 (2013).","DOI":"10.1186\/2046-2530-1-S1-P1"},{"key":"36","doi-asserted-by":"crossref","unstructured":"36) Abdelhamed Z, Vuong SM, Hill L, Shula C, Timms A, Beier D, Campbell K, Mangano FT, Stottmann RW, Goto J. A mutation in Ccdc39<\/i> causes neonatal hydrocephalus with abnormal motile cilia development in mice. Development<\/i>, 145<\/b>, dev154500 (2018).","DOI":"10.1242\/dev.154500"},{"key":"37","doi-asserted-by":"crossref","unstructured":"37) Castleman VH, Romio L, Chodhari R, et al.<\/i> Mutations in radial spoke head protein genes RSPH9 and RSPH4A cause primary ciliary dyskinesia with central-microtubular-pair abnormalities. Am. J. Hum. Genet.<\/i>, 84<\/b>, 197\u2013209 (2009).","DOI":"10.1016\/j.ajhg.2009.01.011"},{"key":"38","doi-asserted-by":"crossref","unstructured":"38) Yoke H, Ueno H, Narita A, Sakai T, Horiuchi K, Shingyoji C, Hamada H, Shinohara K. Rsph4a is essential for the triplet radial spoke head assembly of the mouse motile cilia. PLoS Genet.<\/i>, 16<\/b>, e1008664 (2020).","DOI":"10.1371\/journal.pgen.1008664"},{"key":"39","doi-asserted-by":"crossref","unstructured":"39) Zou W, Lv Y, Liu Z, Xia P, Li H, Jiao J. Loss of Rsph9 causes neonatal hydrocephalus with abnormal development of motile cilia in mice. Sci. Rep.<\/i>, 10<\/b>, 12435 (2020).","DOI":"10.1038\/s41598-020-69447-4"},{"key":"40","doi-asserted-by":"crossref","unstructured":"40) Morimoto Y, Yoshida S, Kinoshita A, Satoh C, Mishima H, Yamaguchi N, Matsuda K, Sakaguchi M, Tanaka T, Komohara Y, Imamura A, Ozawa H, Nakashima M, Kurotaki N, Kishino T, Yoshiura K, Ono S. Nonsense mutation in CFAP43<\/i> causes normal-pressure hydrocephalus with ciliary abnormalities. Neurology<\/i>, 92<\/b>, e2364\u2013e2374 (2019).","DOI":"10.1212\/WNL.0000000000007505"},{"key":"41","doi-asserted-by":"crossref","unstructured":"41) Milara J, Armengot M, Mata M, Morcillo EJ, Cortijo J. Role of adenylate kinase type 7 expression on cilia motility: possible link in primary ciliary dyskinesia. Am. J. Rhinol. Allergy<\/i>, 24<\/b>, 181\u2013185 (2010).","DOI":"10.2500\/ajra.2010.24.3468"},{"key":"42","doi-asserted-by":"crossref","unstructured":"42) Vogel P, Read RW, Hansen GM, Payne BJ, Small D, Sands AT, Zambrowicz BP. Congenital hydrocephalus in genetically engineered mice. Vet. Pathol.<\/i>, 49<\/b>, 166\u2013181 (2012).","DOI":"10.1177\/0300985811415708"},{"key":"43","doi-asserted-by":"crossref","unstructured":"43) Yang HW, Lee S, Berry BC, Yang D, Zheng S, Carroll RS, Park PJ, Johnson MD. A role for mutations in AK9<\/i> and other genes affecting ependymal cells in idiopathic normal pressure hydrocephalus. Proc. Natl. Acad. Sci. U.S.A.<\/i>, 120<\/b>, e2300681120 (2023).","DOI":"10.1073\/pnas.2300681120"},{"key":"44","doi-asserted-by":"crossref","unstructured":"44) Spassky N, Merkle FT, Flames N, Tramontin AD, Garc\u00eda-Verdugo JM, Alvarez-Buylla A. Adult ependymal cells are postmitotic and are derived from radial glial cells during embryogenesis. J. Neurosci.<\/i>, 25<\/b>, 10\u201318 (2005).","DOI":"10.1523\/JNEUROSCI.1108-04.2005"},{"key":"45","doi-asserted-by":"crossref","unstructured":"45) Fuentealba LC, Rompani SB, Parraguez JI, Obernier K, Romero R, Cepko CL, Alvarez-Buylla A. Embryonic origin of postnatal neural stem cells. Cell<\/i>, 161<\/b>, 1644\u20131655 (2015).","DOI":"10.1016\/j.cell.2015.05.041"},{"key":"46","doi-asserted-by":"crossref","unstructured":"46) Ortiz-\u00c1lvarez G, Daclin M, Shihavuddin A, Lansade P, Fortoul A, Faucourt M, Clavreul S, Lalioti M-E, Taraviras S, Hippenmeyer S, Livet J, Meunier A, Genovesio A, Spassky N. Adult neural stem cells and multiciliated ependymal cells share a common lineage regulated by the Geminin family members. Neuron<\/i>, 102<\/b>, 159\u2013172.e7 (2019).","DOI":"10.1016\/j.neuron.2019.01.051"},{"key":"47","doi-asserted-by":"crossref","unstructured":"47) Redmond SA, Figueres-O\u00f1ate M, Obernier K, Nascimento MA, Parraguez JI, L\u00f3pez-Mascaraque L, Fuentealba LC, Alvarez-Buylla A. Development of ependymal and postnatal neural stem cells and their origin from a common embryonic progenitor. Cell Rep.<\/i>, 27<\/b>, 429\u2013441.e3 (2019).","DOI":"10.1016\/j.celrep.2019.01.088"},{"key":"48","doi-asserted-by":"crossref","unstructured":"48) Lokka G, Chantzara A, Lygerou Z, Taraviras S. Ependymal and neural stem cells are close relatives. Stem Cell Reports<\/i>, 20<\/b>, 102574 (2025).","DOI":"10.1016\/j.stemcr.2025.102574"},{"key":"49","doi-asserted-by":"crossref","unstructured":"49) Mori N, Kuwamura M, Tanaka N, Hirano R, Nabe M, Ibuki M, Yamate J. Ccdc85c encoding a protein at apical junctions of radial glia is disrupted in hemorrhagic hydrocephalus (hhy) mice. Am. J. Pathol.<\/i>, 180<\/b>, 314\u2013327 (2012).","DOI":"10.1016\/j.ajpath.2011.09.014"},{"key":"50","doi-asserted-by":"crossref","unstructured":"50) Yamamoto H, Maruo T, Majima T, Ishizaki H, Tanaka-Okamoto M, Miyoshi J, Mandai K, Takai Y. Genetic deletion of afadin causes hydrocephalus by destruction of adherens junctions in radial glial and ependymal cells in the midbrain. PLoS One<\/i>, 8<\/b>, e80356 (2013).","DOI":"10.1371\/journal.pone.0080356"},{"key":"51","doi-asserted-by":"crossref","unstructured":"51) Taylor MD, Poppleton H, Fuller C, Su X, Liu Y, Jensen P, Magdaleno S, Dalton J, Calabrese C, Board J, MacDonald T, Rutka J, Guha A, Gajjar A, Curran T, Gilbertson RJ. Radial glia cells are candidate stem cells of ependymoma. Cancer Cell<\/i>, 8<\/b>, 323\u2013335 (2005).","DOI":"10.1016\/j.ccr.2005.09.001"},{"key":"52","doi-asserted-by":"crossref","unstructured":"52) Herranz-P\u00e9rez V, Nakatani J, Ishii M, Katada T, Garc\u00eda-Verdugo JM, Ohata S. Ependymoma associated protein Zfta is expressed in immature ependymal cells but is not essential for ependymal development in mice. Sci. Rep.<\/i>, 12<\/b>, 1493 (2022).","DOI":"10.1038\/s41598-022-05526-y"},{"key":"53","unstructured":"53) Kardian AS, Sun H, Ippagunta S, et al.<\/i> Dominant clones leverage developmental epigenomic states to drive ependymoma. Nature<\/i>, 652<\/b>, 1027\u20131037 (2026)."},{"key":"54","doi-asserted-by":"crossref","unstructured":"54) Choksi SP, Lauter G, Swoboda P, Roy S. Switching on cilia: transcriptional networks regulating ciliogenesis. Development<\/i>, 141<\/b>, 1427\u20131441 (2014).","DOI":"10.1242\/dev.074666"},{"key":"55","doi-asserted-by":"crossref","unstructured":"55) Kyrousi C, Arbi M, Pilz G-A, Pefani D-E, Lalioti M-E, Ninkovic J, G\u00f6tz M, Lygerou Z, Taraviras S. Mcidas and GemC1 are key regulators for the generation of multiciliated ependymal cells in the adult neurogenic niche. Development<\/i>, 142<\/b>, 3661\u20133674 (2015).","DOI":"10.1242\/dev.126342"},{"key":"56","doi-asserted-by":"crossref","unstructured":"56) Arbi M, Pefani D, Kyrousi C, Lalioti M, Kalogeropoulou A, Papanastasiou AD, Taraviras S, Lygerou Z. GemC1 controls multiciliogenesis in the airway epithelium. EMBO Rep.<\/i>, 17<\/b>, 400\u2013413 (2016).","DOI":"10.15252\/embr.201540882"},{"key":"57","doi-asserted-by":"crossref","unstructured":"57) Boon M, Wallmeier J, Ma L, et al.<\/i> MCIDAS mutations result in a mucociliary clearance disorder with reduced generation of multiple motile cilia. Nat. Commun.<\/i>, 5<\/b>, 4418 (2014).","DOI":"10.1038\/ncomms5418"},{"key":"58","doi-asserted-by":"crossref","unstructured":"58) Ma L, Quigley I, Omran H, Kintner C. Multicilin drives centriole biogenesis via<\/i> E2f proteins. Genes Dev.<\/i>, 28<\/b>, 1461\u20131471 (2014).","DOI":"10.1101\/gad.243832.114"},{"key":"59","doi-asserted-by":"crossref","unstructured":"59) Kim S, Ma L, Shokhirev MN, Quigley I, Kintner C. Multicilin and activated E2f4 induce multiciliated cell differentiation in primary fibroblasts. Sci. Rep.<\/i>, 8<\/b>, 12369 (2018).","DOI":"10.1038\/s41598-018-30791-1"},{"key":"60","doi-asserted-by":"crossref","unstructured":"60) Lewis M, Terr\u00e9 B, Knobel PA, et al.<\/i> GEMC1 and MCIDAS interactions with SWI\/SNF complexes regulate the multiciliated cell-specific transcriptional program. Cell Death Dis.<\/i>, 14<\/b>, 201 (2023).","DOI":"10.1038\/s41419-023-05720-4"},{"key":"61","doi-asserted-by":"crossref","unstructured":"61) Jacquet BV, Salinas-Mondragon R, Liang H, Therit B, Buie JD, Dykstra M, Campbell K, Ostrowski LE, Brody SL, Ghashghaei HT. FoxJ1-dependent gene expression is required for differentiation of radial glia into ependymal cells and a subset of astrocytes in the postnatal brain. Development<\/i>, 136<\/b>, 4021\u20134031 (2009).","DOI":"10.1242\/dev.041129"},{"key":"62","doi-asserted-by":"crossref","unstructured":"62) Hou CC, Li D, Berry BC, Zheng S, Carroll RS, Johnson MD, Yang HW. Heterozygous FOXJ1 mutations cause incomplete ependymal cell differentiation and communicating hydrocephalus. Cell. Mol. Neurobiol.<\/i>, 43<\/b>, 4103\u20134116 (2023).","DOI":"10.1007\/s10571-023-01398-6"},{"key":"63","doi-asserted-by":"crossref","unstructured":"63) Baas D, Meiniel A, Benadiba C, Bonnafe E, Meiniel O, Reith W, Durand B. A deficiency in RFX3 causes hydrocephalus associated with abnormal differentiation of ependymal cells. Eur. J. Neurosci.<\/i>, 24<\/b>, 1020\u20131030 (2006).","DOI":"10.1111\/j.1460-9568.2006.05002.x"},{"key":"64","doi-asserted-by":"crossref","unstructured":"64) Lemeille S, Paschaki M, Baas D, Morl\u00e9 L, Duteyrat J-L, Ait-Lounis A, Barras E, Soulavie F, Jerber J, Thomas J, Zhang Y, Holtzman MJ, Kistler WS, Reith W, Durand B. Interplay of RFX transcription factors 1, 2 and 3 in motile ciliogenesis. Nucleic Acids Res.<\/i>, 48<\/b>, 9019\u20139036 (2020).","DOI":"10.1093\/nar\/gkaa625"},{"key":"65","doi-asserted-by":"crossref","unstructured":"65) El Zein L, Ait-Lounis A, Morl\u00e9 L, Thomas J, Chhin B, Spassky N, Reith W, Durand B. RFX3 governs growth and beating efficiency of motile cilia in mouse and controls the expression of genes involved in human ciliopathies. J. Cell Sci.<\/i>, 122<\/b>, 3180\u20133189 (2009).","DOI":"10.1242\/jcs.048348"},{"key":"66","doi-asserted-by":"crossref","unstructured":"66) Quigley IK, Kintner C. Rfx2 stabilizes Foxj1 binding at chromatin loops to enable multiciliated cell gene expression. PLoS Genet.<\/i>, 13<\/b>, e1006538 (2017).","DOI":"10.1371\/journal.pgen.1006538"},{"key":"67","doi-asserted-by":"crossref","unstructured":"67) Zhao H, Zhu L, Zhu Y, Cao J, Li S, Huang Q, Xu T, Huang X, Yan X, Zhu X. The Cep63 paralogue Deup1 enables massive de novo<\/i> centriole biogenesis for vertebrate multiciliogenesis. Nat. Cell Biol.<\/i>, 15<\/b>, 1434\u20131444 (2013).","DOI":"10.1038\/ncb2880"},{"key":"68","doi-asserted-by":"crossref","unstructured":"68) Klos Dehring DA, Vladar EK, Werner ME, Mitchell JW, Hwang P, Mitchell BJ. Deuterosome-mediated centriole biogenesis. Dev. Cell<\/i>, 27<\/b>, 103\u2013112 (2013).","DOI":"10.1016\/j.devcel.2013.08.021"},{"key":"69","doi-asserted-by":"crossref","unstructured":"69) Tan FE, Vladar EK, Ma L, Fuentealba LC, Hoh R, Espinoza FH, Axelrod JD, Alvarez-Buylla A, Stearns T, Kintner C, Krasnow MA. Myb<\/i> promotes centriole amplification and later steps of the multiciliogenesis program. Development<\/i>, 140<\/b>, 4277\u20134286 (2013).","DOI":"10.1242\/dev.094102"},{"key":"70","doi-asserted-by":"crossref","unstructured":"70) Sorokin SP. Reconstructions of centriole formation and ciliogenesis in mammalian lungs. J. Cell Sci.<\/i>, 3<\/b>, 207\u2013230 (1968).","DOI":"10.1242\/jcs.3.2.207"},{"key":"71","doi-asserted-by":"crossref","unstructured":"71) Al Jord A, Lema\u00eetre A-I, Delgehyr N, Faucourt M, Spassky N, Meunier A. Centriole amplification by mother and daughter centrioles differs in multiciliated cells. Nature<\/i>, 516<\/b>, 104\u2013107 (2014).","DOI":"10.1038\/nature13770"},{"key":"72","doi-asserted-by":"crossref","unstructured":"72) Al Jord A, Shihavuddin A, Servignat d\u2019Aout R, Faucourt M, Genovesio A, Karaiskou A, Sobczak-Th\u00e9pot J, Spassky N, Meunier A. Calibrated mitotic oscillator drives motile ciliogenesis. Science<\/i>, 358<\/b>, 803\u2013806 (2017).","DOI":"10.1126\/science.aan8311"},{"key":"73","doi-asserted-by":"crossref","unstructured":"73) Revinski DR, Zaragosi L-E, Boutin C, Ruiz-Garcia S, Deprez M, Thom\u00e9 V, Rosnet O, Gay A-S, Mercey O, Paquet A, Pons N, Ponzio G, Marcet B, Kodjabachian L, Barbry P. CDC20B is required for deuterosome-mediated centriole production in multiciliated cells. Nat. Commun.<\/i>, 9<\/b>, 4668 (2018).","DOI":"10.1038\/s41467-018-06768-z"},{"key":"74","doi-asserted-by":"crossref","unstructured":"74) Wallmeier J, Al-Mutairi DA, Chen C-T, et al.<\/i> Mutations in CCNO result in congenital mucociliary clearance disorder with reduced generation of multiple motile cilia. Nat. Genet.<\/i>, 46<\/b>, 646\u2013651 (2014).","DOI":"10.1038\/ng.2961"},{"key":"75","doi-asserted-by":"crossref","unstructured":"75) Amirav I, Wallmeier J, Loges NT, Menchen T, Pennekamp P, Mussaffi H, Abitbul R, Avital A, Bentur L, Dougherty GW, Nael E, Lavie M, Olbrich H, Werner C, Kintner C, Omran H. Systematic analysis of CCNO variants in a defined population: Implications for clinical phenotype and differential diagnosis. Hum. Mutat.<\/i>, 37<\/b>, 396\u2013405 (2016).","DOI":"10.1002\/humu.22957"},{"key":"76","doi-asserted-by":"crossref","unstructured":"76) Furey CG, Choi J, Jin SC, et al.<\/i> De novo<\/i> mutation in genes regulating neural stem cell fate in human congenital hydrocephalus. Neuron<\/i>, 99<\/b>, 302\u2013314.e4 (2018).","DOI":"10.1016\/j.neuron.2018.06.019"},{"key":"77","doi-asserted-by":"crossref","unstructured":"77) Rive Le Gouard N, Nicolle R, Lefebvre M, Gelot A, Heide S, Gerasimenko A, Grigorescu R, Derive N, Jouannic J-M, Garel C, Valence S, Quenum-Miraillet G, Chantot-Bastaraud S, Keren B, Heron D, Attie-Bitach T. First reports of fetal SMARCC1 related hydrocephalus. Eur. J. Med. Genet.<\/i>, 66<\/b>, 104797 (2023).","DOI":"10.1016\/j.ejmg.2023.104797"},{"key":"78","doi-asserted-by":"crossref","unstructured":"78) Omiya H, Yamaguchi S, Watanabe T, Kuniya T, Harada Y, Kawaguchi D, Gotoh Y. BMP signaling suppresses Gemc1 expression and ependymal differentiation of mouse telencephalic progenitors. Sci. Rep.<\/i>, 11<\/b>, 613 (2021).","DOI":"10.1038\/s41598-020-79610-6"},{"key":"79","doi-asserted-by":"crossref","unstructured":"79) Bankol\u00e9 A, Srivastava A, Shihavuddin A, Tighanimine K, Faucourt M, Koka V, Weill S, Nemazanyy I, Nelson AJ, Stokes MP, Delgehyr N, Genovesio A, Meunier A, Fumagalli S, Pende M, Spassky N. mTOR controls ependymal cell differentiation by targeting the alternative cell cycle and centrosomal proteins. EMBO Rep.<\/i>, 26<\/b>, 3075\u20133105 (2025).","DOI":"10.1038\/s44319-025-00460-2"},{"key":"80","doi-asserted-by":"crossref","unstructured":"80) Mirzaa GM, Conway RL, Gripp KW, Lerman-Sagie T, Siegel DH, deVries LS, Lev D, Kramer N, Hopkins E, Graham JM Jr, Dobyns WB. Megalencephaly-capillary malformation (MCAP) and megalencephaly-polydactyly-polymicrogyria-hydrocephalus (MPPH) syndromes: Two closely related disorders of brain overgrowth and abnormal brain and body morphogenesis. Am. J. Med. Genet. A.<\/i>, 158A<\/b>, 269\u2013291 (2012).","DOI":"10.1002\/ajmg.a.34402"},{"key":"81","doi-asserted-by":"crossref","unstructured":"81) Roy A, Skibo J, Kalume F, Ni J, Rankin S, Lu Y, Dobyns WB, Mills GB, Zhao JJ, Baker SJ, Millen KJ. Mouse models of human PIK3CA-related brain overgrowth have acutely treatable epilepsy. eLife<\/i>, 4<\/b>, e12703 (2015).","DOI":"10.7554\/eLife.12703"},{"key":"82","doi-asserted-by":"crossref","unstructured":"82) Roy A, Murphy RM, Deng M, MacDonald JW, Bammler TK, Aldinger KA, Glass IA, Millen KJ. PI3K-Yap activity drives cortical gyrification and hydrocephalus in mice. eLife<\/i>, 8<\/b>, e45961 (2019).","DOI":"10.7554\/eLife.45961"},{"key":"83","doi-asserted-by":"crossref","unstructured":"83) Basso M, Mahuzier A, Ali SK, Marty A, Faucourt M, Lennon-Dum\u00e9nil A-M, Srivastava A, Khoury Damaa M, Bankol\u00e9 A, Meunier A, Yamada A, Plastino J, Spassky N, Delgehyr N. Actin-based deformations of the nucleus control mouse multiciliated ependymal cell differentiation. Dev. Cell<\/i>, 60<\/b>, 749\u2013761.e5 (2025).","DOI":"10.1016\/j.devcel.2024.11.008"},{"key":"84","doi-asserted-by":"crossref","unstructured":"84) Nakayama Y, Kohno K. Number and polarity of the ependymal cilia in the central canal of some vertebrates. J. Neurocytol.<\/i>, 3<\/b>, 449\u2013458 (1974).","DOI":"10.1007\/BF01098732"},{"key":"85","doi-asserted-by":"crossref","unstructured":"85) Mirzadeh Z, Han Y-G, Soriano-Navarro M, Garc\u00eda-Verdugo JM, Alvarez-Buylla A. Cilia organize ependymal planar polarity. J. Neurosci.<\/i>, 30<\/b>, 2600\u20132610 (2010).","DOI":"10.1523\/JNEUROSCI.3744-09.2010"},{"key":"86","doi-asserted-by":"crossref","unstructured":"86) Hirota Y, Meunier A, Huang S, Shimozawa T, Yamada O, Kida YS, Inoue M, Ito T, Kato H, Sakaguchi M, Sunabori T, Nakaya M, Nonaka S, Ogura T, Higuchi H, Okano H, Spassky N, Sawamoto K. Planar polarity of multiciliated ependymal cells involves the anterior migration of basal bodies regulated by non-muscle myosin II. Development<\/i>, 137<\/b>, 3037\u20133046 (2010).","DOI":"10.1242\/dev.050120"},{"key":"87","doi-asserted-by":"crossref","unstructured":"87) Brooks ER, Wallingford JB. Multiciliated cells. Curr. Biol.<\/i>, 24<\/b>, R973\u2013R982 (2014).","DOI":"10.1016\/j.cub.2014.08.047"},{"key":"88","doi-asserted-by":"crossref","unstructured":"88) Lim DA, Tramontin AD, Trevejo JM, Herrera DG, Garc\u00eda-Verdugo JM, Alvarez-Buylla A. Noggin antagonizes BMP signaling to create a niche for adult neurogenesis. Neuron<\/i>, 28<\/b>, 713\u2013726 (2000).","DOI":"10.1016\/S0896-6273(00)00148-3"},{"key":"89","doi-asserted-by":"crossref","unstructured":"89) Tong CK, Chen J, Cebri\u00e1n-Silla A, Mirzadeh Z, Obernier K, Guinto CD, Tecott LH, Garc\u00eda-Verdugo JM, Kriegstein A, Alvarez-Buylla A. Axonal control of the adult neural stem cell niche. Cell Stem Cell<\/i>, 14<\/b>, 500\u2013511 (2014).","DOI":"10.1016\/j.stem.2014.01.014"},{"key":"90","doi-asserted-by":"crossref","unstructured":"90) Shi D-L. Planar cell polarity regulators in asymmetric organogenesis during development and disease. J. Genet. Genomics<\/i>, 50<\/b>, 63\u201376 (2023).","DOI":"10.1016\/j.jgg.2022.06.007"},{"key":"91","doi-asserted-by":"crossref","unstructured":"91) Kacker S, Parsad V, Singh N, Hordiichuk D, Alvarez S, Gohar M, Kacker A, Rai SK. Planar cell polarity signaling: Coordinated crosstalk for cell orientation. J. Dev. Biol.<\/i>, 12<\/b>, 12 (2024).","DOI":"10.3390\/jdb12020012"},{"key":"92","doi-asserted-by":"crossref","unstructured":"92) Boutin C, Labedan P, Dimidschstein J, Richard F, Cremer H, Andr\u00e9 P, Yang Y, Montcouquiol M, Goffinet AM, Tissir F. A dual role for planar cell polarity genes in ciliated cells. Proc. Natl. Acad. Sci. U.S.A.<\/i>, 111<\/b>, E3129\u2013E3138 (2014).","DOI":"10.1073\/pnas.1404988111"},{"key":"93","doi-asserted-by":"crossref","unstructured":"93) Ohata S, Herranz-Perez V, Nakatani J, Boletta A, Garcia-Verdugo JM, Alvarez-Buylla A. Mechanosensory genes Pkd1 and Pkd2 contribute to the planar polarization of brain ventricular epithelium. J. Neurosci.<\/i>, 35<\/b>, 11153\u201311168 (2015).","DOI":"10.1523\/JNEUROSCI.0686-15.2015"},{"key":"94","doi-asserted-by":"crossref","unstructured":"94) Tissir F, Qu Y, Montcouquiol M, Zhou L, Komatsu K, Shi D, Fujimori T, Labeau J, Tyteca D, Courtoy P, Poumay Y, Uemura T, Goffinet AM. Lack of cadherins Celsr2 and Celsr3 impairs ependymal ciliogenesis, leading to fatal hydrocephalus. Nat. Neurosci.<\/i>, 13<\/b>, 700\u2013707 (2010).","DOI":"10.1038\/nn.2555"},{"key":"95","doi-asserted-by":"crossref","unstructured":"95) Takagishi M, Sawada M, Ohata S, Asai N, Enomoto A, Takahashi K, Weng L, Ushida K, Ara H, Matsui S, Kaibuchi K, Sawamoto K, Takahashi M. Daple coordinates planar polarized microtubule dynamics in ependymal cells and contributes to hydrocephalus. Cell Rep.<\/i>, 20<\/b>, 960\u2013972 (2017).","DOI":"10.1016\/j.celrep.2017.06.089"},{"key":"96","doi-asserted-by":"crossref","unstructured":"96) Ohata S, Nakatani J, Herranz-P\u00e9rez V, Cheng J, Belinson H, Inubushi T, Snider WD, Garc\u00eda-Verdugo JM, Wynshaw-Boris A, \u00c1lvarez-Buylla A. Loss of Dishevelleds disrupts planar polarity in ependymal motile cilia and results in hydrocephalus. Neuron<\/i>, 83<\/b>, 558\u2013571 (2014).","DOI":"10.1016\/j.neuron.2014.06.022"},{"key":"97","doi-asserted-by":"crossref","unstructured":"97) Vladar EK, Bayly RD, Sangoram AM, Scott MP, Axelrod JD. Microtubules enable the planar cell polarity of airway cilia. Curr. Biol.<\/i>, 22<\/b>, 2203\u20132212 (2012).","DOI":"10.1016\/j.cub.2012.09.046"},{"key":"98","doi-asserted-by":"crossref","unstructured":"98) Kibar Z, Torban E, McDearmid JR, Reynolds A, Berghout J, Mathieu M, Kirillova I, De Marco P, Merello E, Hayes JM, Wallingford JB, Drapeau P, Capra V, Gros P. Mutations in VANGL1<\/i> associated with neural-tube defects. N. Engl. J. Med.<\/i>, 356<\/b>, 1432\u20131437 (2007).","DOI":"10.1056\/NEJMoa060651"},{"key":"99","doi-asserted-by":"crossref","unstructured":"99) Lei Y-P, Zhang T, Li H, Wu B-L, Jin L, Wang H-Y. VANGL2<\/i> mutations in human cranial neural-tube defects. N. Engl. J. Med.<\/i>, 362<\/b>, 2232\u20132235 (2010).","DOI":"10.1056\/NEJMc0910820"},{"key":"100","doi-asserted-by":"crossref","unstructured":"100) Kibar Z, Vogan KJ, Groulx N, Justice MJ, Underhill DA, Gros P. Ltap, a mammalian homolog of Drosophila Strabismus\/Van Gogh, is altered in the mouse neural tube mutant Loop-tail. Nat. Genet.<\/i>, 28<\/b>, 251\u2013255 (2001).","DOI":"10.1038\/90081"},{"key":"101","doi-asserted-by":"crossref","unstructured":"101) Zhang F, Li S, Wu H, Chen S. Cryo-EM structure and oligomerization of the human planar cell polarity core protein Vangl1. Nat. Commun.<\/i>, 16<\/b>, 135 (2025).","DOI":"10.1038\/s41467-024-55397-2"},{"key":"102","doi-asserted-by":"crossref","unstructured":"102) Jussila M, Boswell CW, Griffiths NW, Pumputis PG, Ciruna B. Live imaging and conditional disruption of native PCP activity using endogenously tagged zebrafish sfGFP-Vangl2. Nat. Commun.<\/i>, 13<\/b>, 5598 (2022).","DOI":"10.1038\/s41467-022-33322-9"},{"key":"103","doi-asserted-by":"crossref","unstructured":"103) Ying J, Yang Y, Zhang X, Dong Z, Chen B. Stearoylation cycle regulates the cell surface distribution of the PCP protein Vangl2. Proc. Natl. Acad. Sci. U.S.A.<\/i>, 121<\/b>, e2400569121 (2024).","DOI":"10.1073\/pnas.2400569121"},{"key":"104","doi-asserted-by":"crossref","unstructured":"104) Allache R, De Marco P, Merello E, Capra V, Kibar Z. Role of the planar cell polarity gene CELSR1<\/i> in neural tube defects and caudal agenesis. Birth Defects Res. A Clin. Mol. Teratol.<\/i>, 94<\/b>, 176\u2013181 (2012).","DOI":"10.1002\/bdra.23002"},{"key":"105","doi-asserted-by":"crossref","unstructured":"105) Chen Z, Lei Y, Cao X, Zheng Y, Wang F, Bao Y, Peng R, Finnell RH, Zhang T, Wang H. Genetic analysis of Wnt\/PCP genes in neural tube defects. BMC Med. Genomics<\/i>, 11<\/b>, 38 (2018).","DOI":"10.1186\/s12920-018-0355-9"},{"key":"106","doi-asserted-by":"crossref","unstructured":"106) Bandekar SJ, Garbett K, Kordon SP, Dintzner EE, Li J, Shearer T, Sando RC, Ara\u00e7 D. Structural basis for regulation of CELSR1 by a compact module in its extracellular region. Nat. Commun.<\/i>, 16<\/b>, 3972 (2025).","DOI":"10.1038\/s41467-025-59319-8"},{"key":"107","doi-asserted-by":"crossref","unstructured":"107) Ekici AB, Hilfinger D, Jatzwauk M, Thiel CT, Wenzel D, Lorenz I, Boltshauser E, Goecke TW, Staatz G, Morris-Rosendahl DJ, Sticht H, Hehr U, Reis A, Rauch A. Disturbed Wnt signalling due to a mutation in CCDC88C causes an autosomal recessive non-syndromic hydrocephalus with medial diverticulum. Mol. Syndromol.<\/i>, 1<\/b>, 99\u2013112 (2010).","DOI":"10.1159\/000319859"},{"key":"108","doi-asserted-by":"crossref","unstructured":"108) Drielsma A, Jalas C, Simonis N, D\u00e9sir J, Simanovsky N, Pirson I, Elpeleg O, Abramowicz M, Edvardson S. Two novel CCDC88C mutations confirm the role of DAPLE in autosomal recessive congenital hydrocephalus. J. Med. Genet.<\/i>, 49<\/b>, 708\u2013712 (2012).","DOI":"10.1136\/jmedgenet-2012-101190"},{"key":"109","doi-asserted-by":"crossref","unstructured":"109) Marguet F, Vezain M, Marcorelles P, Audebert-Bellanger S, Cassinari K, Drouot N, Chambon P, Gonzalez BJ, Horowitz A, Laquerriere A, Saugier-Veber P. Neuropathological hallmarks of fetal hydrocephalus linked to CCDC88C pathogenic variants. Acta Neuropathol. Commun.<\/i>, 9<\/b>, 104 (2021).","DOI":"10.1186\/s40478-021-01207-5"},{"key":"110","doi-asserted-by":"crossref","unstructured":"110) De Marco P, Merello E, Rossi A, Piatelli G, Cama A, Kibar Z, Capra V. FZD6<\/i> is a novel gene for human neural tube defects. Hum. Mutat.<\/i>, 33<\/b>, 384\u2013390 (2012).","DOI":"10.1002\/humu.21643"},{"key":"111","doi-asserted-by":"crossref","unstructured":"111) Kilander MBC, Petersen J, Andressen KW, Ganji RS, Levy FO, Schuster J, Dahl N, Bryja V, Schulte G. Disheveled regulates precoupling of heterotrimeric G proteins to Frizzled 6. FASEB J.<\/i>, 28<\/b>, 2293\u20132305 (2014).","DOI":"10.1096\/fj.13-246363"},{"key":"112","doi-asserted-by":"crossref","unstructured":"112) Zhang Z, Lin X, Wei L, Wu Y, Xu L, Wu L, Wei X, Zhao S, Zhu X, Xu F. A framework for Frizzled-G protein coupling and implications to the PCP signaling pathways. Cell Discov.<\/i>, 10<\/b>, 3 (2024).","DOI":"10.1038\/s41421-023-00627-y"},{"key":"113","doi-asserted-by":"crossref","unstructured":"113) Wang Y, Guo N, Nathans J. The role of Frizzled3<\/i> and Frizzled6<\/i> in neural tube closure and in the planar polarity of inner-ear sensory hair cells. J. Neurosci.<\/i>, 26<\/b>, 2147\u20132156 (2006).","DOI":"10.1523\/JNEUROSCI.4698-05.2005"},{"key":"114","doi-asserted-by":"crossref","unstructured":"114) Stuebner S, Faus-Kessler T, Fischer T, Wurst W, Prakash N. Fzd3<\/i> and Fzd6<\/i> deficiency results in a severe midbrain morphogenesis defect. Dev. Dyn.<\/i>, 239<\/b>, 246\u2013260 (2010).","DOI":"10.1002\/dvdy.22127"},{"key":"115","doi-asserted-by":"crossref","unstructured":"115) Pan J, You Y, Huang T, Brody SL. RhoA-mediated apical actin enrichment is required for ciliogenesis and promoted by Foxj1. J. Cell Sci.<\/i>, 120<\/b>, 1868\u20131876 (2007).","DOI":"10.1242\/jcs.005306"},{"key":"116","doi-asserted-by":"crossref","unstructured":"116) Newell-Litwa KA, Horwitz R, Lamers ML. Non-muscle myosin II in disease: mechanisms and therapeutic opportunities. Dis. Model. Mech.<\/i>, 8<\/b>, 1495\u20131515 (2015).","DOI":"10.1242\/dmm.022103"},{"key":"117","doi-asserted-by":"crossref","unstructured":"117) Wiegel J, Helmst\u00e4dter M, Walz G, Bergen MD. Spontaneous calcium bursts organize the apical actin cytoskeleton of multiciliated cells. Int. J. Mol. Sci.<\/i>, 26<\/b>, 2507 (2025).","DOI":"10.3390\/ijms26062507"},{"key":"118","doi-asserted-by":"crossref","unstructured":"118) Fuertes-Alvarez S, Maeso-Alonso L, Villoch-Fernandez J, Wildung M, Martin-Lopez M, Marshall C, Villena-Cortes AJ, Diez-Prieto I, Pietenpol JA, Tissir F, Liz\u00e9 M, Marques MM, Marin MC. p73 regulates ependymal planar cell polarity by modulating actin and microtubule cytoskeleton. Cell Death Dis.<\/i>, 9<\/b>, 1183 (2018).","DOI":"10.1038\/s41419-018-1205-6"},{"key":"119","doi-asserted-by":"crossref","unstructured":"119) Fujitani M, Sato R, Yamashita T. Loss of p73 in ependymal cells during the perinatal period leads to aqueductal stenosis. Sci. Rep.<\/i>, 7<\/b>, 12007 (2017).","DOI":"10.1038\/s41598-017-12105-z"},{"key":"120","doi-asserted-by":"crossref","unstructured":"120) Zimmermann D, Kovar DR. Feeling the force: formin\u2019s role in mechanotransduction. Curr. Opin. Cell Biol.<\/i>, 56<\/b>, 130\u2013140 (2019).","DOI":"10.1016\/j.ceb.2018.12.008"},{"key":"121","doi-asserted-by":"crossref","unstructured":"121) Janke C, Magiera MM. The tubulin code and its role in controlling microtubule properties and functions. Nat. Rev. Mol. Cell Biol.<\/i>, 21<\/b>, 307\u2013326 (2020).","DOI":"10.1038\/s41580-020-0214-3"},{"key":"122","doi-asserted-by":"crossref","unstructured":"122) Bosch Grau M, Gonzalez Curto G, Rocha C, Magiera MM, Marques Sousa P, Giordano T, Spassky N, Janke C. Tubulin glycylases and glutamylases have distinct functions in stabilization and motility of ependymal cilia. J. Cell Biol.<\/i>, 202<\/b>, 441\u2013451 (2013).","DOI":"10.1083\/jcb.201305041"},{"key":"123","doi-asserted-by":"crossref","unstructured":"123) Kubo T, Oda T. Electrostatic interaction between polyglutamylated tubulin and the nexin\u2013dynein regulatory complex regulates flagellar motility. Mol. Biol. Cell<\/i>, 28<\/b>, 2260\u20132266 (2017).","DOI":"10.1091\/mbc.e17-05-0285"},{"key":"124","doi-asserted-by":"crossref","unstructured":"124) Kubo T, Sasaki R, Oda T. Tubulin glycylation controls ciliary motility through modulation of outer-arm dyneins. Mol. Biol. Cell<\/i>, 35<\/b>, ar90 (2024).","DOI":"10.1091\/mbc.E24-04-0154"},{"key":"125","doi-asserted-by":"crossref","unstructured":"125) Lee JE, Silhavy JL, Zaki MS, et al.<\/i> CEP41 is mutated in Joubert syndrome and is required for tubulin glutamylation at the cilium. Nat. Genet.<\/i>, 44<\/b>, 193\u2013199 (2012).","DOI":"10.1038\/ng.1078"},{"key":"126","doi-asserted-by":"crossref","unstructured":"126) He K, Ma X, Xu T, Li Y, Hodge A, Zhang Q, Torline J, Huang Y, Zhao J, Ling K, Hu J. Axoneme polyglutamylation regulated by Joubert syndrome protein ARL13B controls ciliary targeting of signaling molecules. Nat. Commun.<\/i>, 9<\/b>, 3310 (2018).","DOI":"10.1038\/s41467-018-05867-1"},{"key":"127","doi-asserted-by":"crossref","unstructured":"127) He K, Sun X, Chen C, Luc S, Robichaud JH, Zhang Y, Huang Y, Ji B, Ku P-I, Subramanian R, Ling K, Hu J. Non-canonical CDK6 activity promotes cilia disassembly by suppressing axoneme polyglutamylation. J. Cell Biol.<\/i>, 224<\/b>, e202405170 (2025).","DOI":"10.1083\/jcb.202405170"},{"key":"128","doi-asserted-by":"crossref","unstructured":"128) Takagishi M, Esaki N, Takahashi K, Takahashi M. Cytoplasmic dynein functions in planar polarization of basal bodies within ciliated cells. iScience<\/i>, 23<\/b>, 101213 (2020).","DOI":"10.1016\/j.isci.2020.101213"},{"key":"129","doi-asserted-by":"crossref","unstructured":"129) Nakayama S, Yano T, Namba T, Konishi S, Takagishi M, Herawati E, Nishida T, Imoto Y, Ishihara S, Takahashi M, Furuta K, Oiwa K, Tamura A, Tsukita S. Planar cell polarity induces local microtubule bundling for coordinated ciliary beating. J. Cell Biol.<\/i>, 220<\/b>, e202010034 (2021).","DOI":"10.1083\/jcb.202010034"},{"key":"130","doi-asserted-by":"crossref","unstructured":"130) Takagishi M, Yue Y, Gray RS, Verhey KJ, Wallingford JB. Motor protein Kif6 regulates cilia motility and polarity in brain ependymal cells. Dis. Model. Mech.<\/i>, 17<\/b>, dmm050137 (2024).","DOI":"10.1242\/dmm.050137"},{"key":"131","doi-asserted-by":"crossref","unstructured":"131) Li L, Ran J. Regulation of ciliary homeostasis by intraflagellar transport-independent kinesins. Cell Death Dis.<\/i>, 15<\/b>, 47 (2024).","DOI":"10.1038\/s41419-024-06428-9"},{"key":"132","doi-asserted-by":"crossref","unstructured":"132) Konjikusic MJ, Yeetong P, Boswell CW, Lee C, Roberson EC, Ittiwut R, Suphapeetiporn K, Ciruna B, Gurnett CA, Wallingford JB, Shotelersuk V, Gray RS. Mutations in Kinesin family member 6 reveal specific role in ependymal cell ciliogenesis and human neurological development. PLoS Genet.<\/i>, 14<\/b>, e1007817 (2018).","DOI":"10.1371\/journal.pgen.1007817"},{"key":"133","doi-asserted-by":"crossref","unstructured":"133) Pan X, Fang C, Shen C, Li X, Xie L, Li L, Huang S, Yan X, Zhu X. Directional ciliary beats across epithelia require Ccdc57-mediated coupling between axonemal orientation and basal body polarity. Nat. Commun.<\/i>, 15<\/b>, 10249 (2024).","DOI":"10.1038\/s41467-024-54766-1"},{"key":"134","doi-asserted-by":"crossref","unstructured":"134) Guirao B, Meunier A, Mortaud S, Aguilar A, Corsi J-M, Strehl L, Hirota Y, Desoeuvre A, Boutin C, Han Y-G, Mirzadeh Z, Cremer H, Montcouquiol M, Sawamoto K, Spassky N. Coupling between hydrodynamic forces and planar cell polarity orients mammalian motile cilia. Nat. Cell Biol.<\/i>, 12<\/b>, 341\u2013350 (2010).","DOI":"10.1038\/ncb2040"}],"container-title":["Biological and Pharmaceutical Bulletin"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.jstage.jst.go.jp\/article\/bpb\/49\/9\/49_b26-00284\/_pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,9,5]],"date-time":"2026-09-05T03:27:56Z","timestamp":1788578876000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.jstage.jst.go.jp\/article\/bpb\/49\/9\/49_b26-00284\/_article"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,9,1]]},"references-count":134,"journal-issue":{"issue":"9","published-print":{"date-parts":[[2026]]}},"URL":"https:\/\/doi.org\/10.1248\/bpb.b26-00284","relation":{},"ISSN":["0918-6158","1347-5215"],"issn-type":[{"value":"0918-6158","type":"print"},{"value":"1347-5215","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,9,1]]},"article-number":"b26-00284"}}