Gene Symbol: Celsr1
Description: cadherin, EGF LAG seven-pass G-type receptor 1
Alias: Adgrc1, Crsh, Scy, crash, cadherin EGF LAG seven-pass G-type receptor 1, cadherin, EGF LAG seven-pass G-type receptor 1 (flamingo homolog, Drosophila), seven-pass transmembrane receptor
Species: mouse
Products:     Celsr1

Top Publications

  1. Qu Y, Glasco D, Zhou L, Sawant A, Ravni A, Fritzsch B, et al. Atypical cadherins Celsr1-3 differentially regulate migration of facial branchiomotor neurons in mice. J Neurosci. 2010;30:9392-401 pubmed publisher
    ..Here, we investigated the role of cadherins Celsr1-3, and Fzd3 in FBM neuron migration in mice...
  2. GARCIA GARCIA M, Shibata M, Anderson K. Chato, a KRAB zinc-finger protein, regulates convergent extension in the mouse embryo. Development. 2008;135:3053-62 pubmed publisher
  3. Formstone C, Moxon C, Murdoch J, Little P, Mason I. Basal enrichment within neuroepithelia suggests novel function(s) for Celsr1 protein. Mol Cell Neurosci. 2010;44:210-22 pubmed publisher
    A characteristic of the 7TM-cadherins, Flamingo and Celsr1, is their asymmetric protein distribution and polarized activity at neighboring epithelial cell interfaces along defined axes of planar cell polarity...
  4. Yates L, Schnatwinkel C, Murdoch J, Bogani D, Formstone C, Townsend S, et al. The PCP genes Celsr1 and Vangl2 are required for normal lung branching morphogenesis. Hum Mol Genet. 2010;19:2251-67 pubmed publisher
    ..Here we show that mutations in the planar cell polarity (PCP) genes Celsr1 and Vangl2 lead to disrupted lung development and defects in lung architecture...
  5. Curtin J, Quint E, Tsipouri V, Arkell R, Cattanach B, Copp A, et al. Mutation of Celsr1 disrupts planar polarity of inner ear hair cells and causes severe neural tube defects in the mouse. Curr Biol. 2003;13:1129-33 pubmed
    ..We show that these mutants, spin cycle and crash, carry independent missense mutations within the coding region of Celsr1, encoding a large protocadherin molecule [1]...
  6. Montcouquiol M, Sans N, Huss D, Kach J, Dickman J, Forge A, et al. Asymmetric localization of Vangl2 and Fz3 indicate novel mechanisms for planar cell polarity in mammals. J Neurosci. 2006;26:5265-75 pubmed
    ..Together, our data support the idea that protein asymmetry plays an important role in the development of PCP, but the colocalization and interaction of Fz3 and Vangl2 suggests that novel PCP mechanisms exist in vertebrates. ..
  7. Paudyal A, Damrau C, Patterson V, Ermakov A, Formstone C, Lalanne Z, et al. The novel mouse mutant, chuzhoi, has disruption of Ptk7 protein and exhibits defects in neural tube, heart and lung development and abnormal planar cell polarity in the ear. BMC Dev Biol. 2010;10:87 pubmed publisher
    ..This mutant provides a useful tool to allow future studies aimed at understanding the molecular function of Ptk7. ..
  8. Formstone C, Little P. The flamingo-related mouse Celsr family (Celsr1-3) genes exhibit distinct patterns of expression during embryonic development. Mech Dev. 2001;109:91-4 pubmed
    We have isolated cDNAs for three members of a family of seven-pass transmembrane cadherins in mouse (Celsr1, 2 and 3)...
  9. Ybot Gonzalez P, Savery D, Gerrelli D, Signore M, Mitchell C, Faux C, et al. Convergent extension, planar-cell-polarity signalling and initiation of mouse neural tube closure. Development. 2007;134:789-99 pubmed
    ..These findings identify a cell-autonomous defect of convergent extension, requiring PCP signalling via RhoA-Rho kinase, during the development of severe neural tube defects in the mouse. ..

More Information


  1. Devenport D, Fuchs E. Planar polarization in embryonic epidermis orchestrates global asymmetric morphogenesis of hair follicles. Nat Cell Biol. 2008;10:1257-68 pubmed publisher
    ..Hair follicle initiation coincides with asymmetric redistribution of Vangl2, Celsr1 and Fzd6 within the embryonic epidermal basal layer...
  2. Tissir F, Qu Y, Montcouquiol M, Zhou L, Komatsu K, Shi D, et al. Lack of cadherins Celsr2 and Celsr3 impairs ependymal ciliogenesis, leading to fatal hydrocephalus. Nat Neurosci. 2010;13:700-7 pubmed publisher
    ..Our findings suggest that planar cell polarity signaling is involved in ependymal cilia development and in the pathophysiology of hydrocephalus, with possible implications in other ciliopathies. ..
  3. Panousopoulou E, Hobbs C, Mason I, Green J, Formstone C. Epiboly generates the epidermal basal monolayer and spreads the nascent mammalian skin to enclose the embryonic body. J Cell Sci. 2016;129:1915-27 pubmed publisher
    ..monolayer formation and epidermal spreading are delayed in Crash mice, which possess a dominant mutation in Celsr1, an orthologue of the core planar cell polarity (PCP) Drosophila protein Flamingo (also known as Stan)...
  4. Zhao T, Gan Q, Stokes A, Lassiter R, Wang Y, Chan J, et al. ?-catenin regulates Pax3 and Cdx2 for caudal neural tube closure and elongation. Development. 2014;141:148-57 pubmed publisher
    ..Thus, ?-catenin signaling is required for caudal neural tube closure and elongation, acting through the transcriptional regulation of key target genes in the PNP. ..
  5. Kadzik R, Cohen E, Morley M, Stewart K, Lu M, Morrisey E. Wnt ligand/Frizzled 2 receptor signaling regulates tube shape and branch-point formation in the lung through control of epithelial cell shape. Proc Natl Acad Sci U S A. 2014;111:12444-9 pubmed publisher
    ..This study demonstrates the importance of Wnt/Fzd2 signaling in promoting and maintaining changes in epithelial cell shape that affect development of a branching network. ..
  6. Glasco D, Pike W, Qu Y, Reustle L, Misra K, Di Bonito M, et al. The atypical cadherin Celsr1 functions non-cell autonomously to block rostral migration of facial branchiomotor neurons in mice. Dev Biol. 2016;417:40-9 pubmed publisher
    ..Although several Wnt/Planar Cell Polarity (PCP) components are required for FBM neuron migration, only Celsr1, an atypical cadherin, regulates the direction of migration in mice...
  7. Shi D, Komatsu K, Hirao M, Toyooka Y, Koyama H, Tissir F, et al. Celsr1 is required for the generation of polarity at multiple levels of the mouse oviduct. Development. 2014;141:4558-68 pubmed publisher
    ..oviduct and report that multilevel polarities of the oviduct are regulated by a planar cell polarity (PCP) gene, Celsr1. In the epithelium, Celsr1 is concentrated in the specific cellular boundaries perpendicular to the ovary-uterus ..
  8. Fong K, Hufnagel R, Khadka V, Corley M, Maunakea A, Fogelgren B, et al. A mutation in the tuft mouse disrupts TET1 activity and alters the expression of genes that are crucial for neural tube closure. Dis Model Mech. 2016;9:585-96 pubmed publisher
    ..These results indicate that TET1 is an epigenetic determinant for regulating genes that are crucial to closure of the anterior neural tube and its mutation has implications to craniofacial development, as presented by the tuft mouse. ..
  9. Cetera M, Leybova L, Woo F, Deans M, Devenport D. Planar cell polarity-dependent and independent functions in the emergence of tissue-scale hair follicle patterns. Dev Biol. 2017;428:188-203 pubmed publisher
  10. Vladar E, Bayly R, Sangoram A, Scott M, Axelrod J. Microtubules enable the planar cell polarity of airway cilia. Curr Biol. 2012;22:2203-12 pubmed publisher
  11. Hadjantonakis A, Formstone C, Little P. mCelsr1 is an evolutionarily conserved seven-pass transmembrane receptor and is expressed during mouse embryonic development. Mech Dev. 1998;78:91-5 pubmed
    ..We observe segmentally-restricted gene expression in the developing hindbrain and in the spinal cord dynamic dorso-ventrally restricted 'stripes' of expression. ..
  12. Tissir F, De Backer O, Goffinet A, Lambert de Rouvroit C. Developmental expression profiles of Celsr (Flamingo) genes in the mouse. Mech Dev. 2002;112:157-60 pubmed
    ..The three Flamingo gene orthologs in man and rodents are named, respectively, CELSR1-3 and Celsr1-3. Celsr1 and 2 are expressed during early development, in the brain and epithelia...
  13. Shima Y, Copeland N, Gilbert D, Jenkins N, Chisaka O, Takeichi M, et al. Differential expression of the seven-pass transmembrane cadherin genes Celsr1-3 and distribution of the Celsr2 protein during mouse development. Dev Dyn. 2002;223:321-32 pubmed
    ..results of our study of its embryonic and postnatal expression patterns together with those of two other paralogs, Celsr1 and Celsr3...
  14. Murdoch J, Damrau C, Paudyal A, Bogani D, Wells S, Greene N, et al. Genetic interactions between planar cell polarity genes cause diverse neural tube defects in mice. Dis Model Mech. 2014;7:1153-63 pubmed publisher
    ..produced in double heterozygotes, we bred mice with all three pairwise combinations of Vangl2(Lp), Scrib(Crc) and Celsr1(Crsh) mutations, the most intensively studied PCP mutants...
  15. Wang Y, Williams J, Rattner A, Wu S, Bassuk A, Goffinet A, et al. Patterning of papillae on the mouse tongue: A system for the quantitative assessment of planar cell polarity signaling. Dev Biol. 2016;419:298-310 pubmed publisher
    ..of lingual papillae are under the control of the core planar cell polarity (PCP) genes Vangl1, Vangl2, and Celsr1. Using K14-Cre and Nkx2...
  16. Wang Y, Baeyens N, Corti F, Tanaka K, Fang J, Zhang J, et al. Syndecan 4 controls lymphatic vasculature remodeling during mouse embryonic development. Development. 2016;143:4441-4451 pubmed
    ..SDC4 thus controls flow-induced LEC polarization via regulation of VANGL2 expression. ..
  17. Minegishi K, Hashimoto M, Ajima R, Takaoka K, Shinohara K, Ikawa Y, et al. A Wnt5 Activity Asymmetry and Intercellular Signaling via PCP Proteins Polarize Node Cells for Left-Right Symmetry Breaking. Dev Cell. 2017;40:439-452.e4 pubmed publisher
  18. Hermle T, Guida M, Beck S, Helmstädter S, Simons M. Drosophila ATP6AP2/VhaPRR functions both as a novel planar cell polarity core protein and a regulator of endosomal trafficking. EMBO J. 2013;32:245-59 pubmed publisher
    ..Together, our results suggest two roles for VhaPRR, one for PCP and another in endosomal trafficking. This dual function establishes VhaPRR as a key factor in epithelial morphogenesis...
  19. Duncan J, Stoller M, Francl A, Tissir F, Devenport D, Deans M. Celsr1 coordinates the planar polarity of vestibular hair cells during inner ear development. Dev Biol. 2017;423:126-137 pubmed publisher
    ..coordinated between neighboring cells by core PCP proteins including the large adhesive G-protein coupled receptor Celsr1. We show that mice lacking Celsr1 have vestibular behavioral phenotypes including circling...
  20. Gegg M, Böttcher A, Burtscher I, Hasenoeder S, Van Campenhout C, Aichler M, et al. Flattop regulates basal body docking and positioning in mono- and multiciliated cells. elife. 2014;3: pubmed publisher
    ..Taken together, the identification of novel BB/spindle positioning components as potential mediators of PCP signaling might have broader implications for other cell types, ciliary disease, and asymmetric cell division. ..
  21. Devenport D, Oristian D, Heller E, Fuchs E. Mitotic internalization of planar cell polarity proteins preserves tissue polarity. Nat Cell Biol. 2011;13:893-902 pubmed publisher
    ..A cytoplasmic dileucine motif governs mitotic internalization of atypical cadherin Celsr1, which recruits Vang2 and Fzd6 to endosomes...
  22. Crompton L, Du Roure C, Rodriguez T. Early embryonic expression patterns of the mouse Flamingo and Prickle orthologues. Dev Dyn. 2007;236:3137-43 pubmed
    ..Here, we show that the murine Flamingo orthologues Celsr1-3 and the Prickle orthologues Prickle1, Prickle2, and Testin have dynamic patterns of expression during ..
  23. Yates L, Schnatwinkel C, Hazelwood L, Chessum L, Paudyal A, Hilton H, et al. Scribble is required for normal epithelial cell-cell contacts and lumen morphogenesis in the mammalian lung. Dev Biol. 2013;373:267-80 pubmed publisher
    ..of Scrib knockdown lungs shows that Scrib does not affect bud bifurcation, as previously shown for the PCP protein Celsr1, but is required to maintain epithelial cohesion...
  24. Boutin C, Labedan P, Dimidschstein J, Richard F, Cremer H, Andre P, et al. A dual role for planar cell polarity genes in ciliated cells. Proc Natl Acad Sci U S A. 2014;111:E3129-38 pubmed publisher
    ..Vangl2) organizes multicilia in individual cells (single-cell polarity), whereas the second signal, governed by Celsr1, Fzd3, and Vangl2, coordinates polarity between cells in both radial progenitors and ependymal cells (tissue ..
  25. Mahaffey J, Grego Bessa J, Liem K, Anderson K. Cofilin and Vangl2 cooperate in the initiation of planar cell polarity in the mouse embryo. Development. 2013;140:1262-71 pubmed publisher
    ..We propose that Vangl2 and cofilin cooperate to target Rab11(+) vesicles containing PCP proteins to the apical membrane during the initiation of planar cell polarity. ..
  26. Luxenburg C, Heller E, Pasolli H, Chai S, Nikolova M, Stokes N, et al. Wdr1-mediated cell shape dynamics and cortical tension are essential for epidermal planar cell polarity. Nat Cell Biol. 2015;17:592-604 pubmed publisher
    ..Our findings suggest intriguing evolutionary parallels but mechanistic modifications to the distal wing hinge-mediated mechanical forces that drive cell shape change and orient PCP in the Drosophila wing disc. ..
  27. Ravni A, Qu Y, Goffinet A, Tissir F. Planar cell polarity cadherin Celsr1 regulates skin hair patterning in the mouse. J Invest Dermatol. 2009;129:2507-9 pubmed publisher
  28. Brzóska H, d Esposito A, Kolatsi Joannou M, Patel V, Igarashi P, Lei Y, et al. Planar cell polarity genes Celsr1 and Vangl2 are necessary for kidney growth, differentiation, and rostrocaudal patterning. Kidney Int. 2016;90:1274-1284 pubmed publisher
    ..Here we show that Celsr1, a planar cell polarity (PCP) gene implicated in neural tube morphogenesis, is required for ureteric tree growth ..
  29. Hadjantonakis A, Sheward W, Harmar A, de Galan L, Hoovers J, Little P. Celsr1, a neural-specific gene encoding an unusual seven-pass transmembrane receptor, maps to mouse chromosome 15 and human chromosome 22qter. Genomics. 1997;45:97-104 pubmed
    We have identified Celsr1, a gene that encodes a developmentally regulated vertebrate seven-pass transmembrane protein...
  30. Glasco D, Sittaramane V, Bryant W, Fritzsch B, Sawant A, Paudyal A, et al. The mouse Wnt/PCP protein Vangl2 is necessary for migration of facial branchiomotor neurons, and functions independently of Dishevelled. Dev Biol. 2012;369:211-22 pubmed publisher
    ..Interestingly, genetic-interaction experiments suggest that rostral FBM neuron migration, which is normally suppressed, depends upon Dvl function. ..
  31. Caddy J, Wilanowski T, Darido C, Dworkin S, Ting S, Zhao Q, et al. Epidermal wound repair is regulated by the planar cell polarity signaling pathway. Dev Cell. 2010;19:138-47 pubmed publisher
    ..Mice carrying mutant alleles of PCP genes Vangl2, Celsr1, PTK7, and Scrb1, and the transcription factor Grhl3, interact genetically, exhibiting failed wound healing, ..
  32. Ren D, Kelly M, Kim S, Grimsley Myers C, Chi F, Chen P. Testin interacts with vangl2 genetically to regulate inner ear sensory cell orientation and the normal development of the female reproductive tract in mice. Dev Dyn. 2013;242:1454-65 pubmed publisher
    ..Furthermore, its genetic interaction with Vangl2 implicated it as a potential molecular link, responsible for mediating the role of Vangl2-containing membranous PCP complexes in directing morphologic polarization. ..