Gene Symbol: Vangl2
Description: VANGL planar cell polarity protein 2
Alias: Ltap, vang-like protein 2, loop tail associated protein, van Gogh-like protein 2, vang-like 2 (van gogh, Drosophila)
Species: rat
Products:     Vangl2

Top Publications

  1. Henderson D, Conway S, Greene N, Gerrelli D, Murdoch J, Anderson R, et al. Cardiovascular defects associated with abnormalities in midline development in the Loop-tail mouse mutant. Circ Res. 2001;89:6-12 pubmed
    ..We suggest that the double-sided arch arises as a primary defect in the Lp mutant, unrelated to the alignment defects, perhaps reflecting a role for the (as-yet-unknown) Lp gene in maintenance/regression of the aortic arch system. ..
  2. Wang J, Hamblet N, Mark S, Dickinson M, Brinkman B, Segil N, et al. Dishevelled genes mediate a conserved mammalian PCP pathway to regulate convergent extension during neurulation. Development. 2006;133:1767-78 pubmed
    ..Dvl2 genetically interacts with Loop-tail, a point mutation in the mammalian PCP gene Vangl2, during neurulation...
  3. Murdoch J, Doudney K, Paternotte C, Copp A, Stanier P. Severe neural tube defects in the loop-tail mouse result from mutation of Lpp1, a novel gene involved in floor plate specification. Hum Mol Genet. 2001;10:2593-601 pubmed
    ..Loss of Lpp1 function disrupts neurulation by permitting more extensive floor plate induction by Shh, thereby inhibiting midline bending of the neural plate during initiation of neurulation. ..
  4. 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...
  5. Wang Y, Guo N, Nathans J. The role of Frizzled3 and Frizzled6 in neural tube closure and in the planar polarity of inner-ear sensory hair cells. J Neurosci. 2006;26:2147-56 pubmed
    ..b>Vangl2, one of two mammalian homologs of the Drosophila planar cell polarity (PCP) gene van Gogh/Strabismus, is also ..
  6. Ross A, May Simera H, Eichers E, Kai M, Hill J, Jagger D, et al. Disruption of Bardet-Biedl syndrome ciliary proteins perturbs planar cell polarity in vertebrates. Nat Genet. 2005;37:1135-40 pubmed
    ..Furthermore, we identify genetic interactions between BBS genes and a PCP gene in both mouse (Ltap, also called Vangl2) and zebrafish (vangl2)...
  7. Yamamoto S, Nishimura O, Misaki K, Nishita M, Minami Y, Yonemura S, et al. Cthrc1 selectively activates the planar cell polarity pathway of Wnt signaling by stabilizing the Wnt-receptor complex. Dev Cell. 2008;15:23-36 pubmed publisher
    ..Although Cthrc1 null mutant mice appeared normal, the introduction of a heterozygous mutation of a PCP gene, Vangl2, resulted in abnormalities characteristic of PCP mutants...
  8. Etheridge S, Ray S, Li S, Hamblet N, Lijam N, Tsang M, et al. Murine dishevelled 3 functions in redundant pathways with dishevelled 1 and 2 in normal cardiac outflow tract, cochlea, and neural tube development. PLoS Genet. 2008;4:e1000259 pubmed publisher
    ..the organ of Corti, a phenotype that was enhanced with the additional loss of a single allele of the PCP component Vangl2/Ltap (LtapLp/+)...
  9. Satoh W, Matsuyama M, Takemura H, Aizawa S, Shimono A. Sfrp1, Sfrp2, and Sfrp5 regulate the Wnt/beta-catenin and the planar cell polarity pathways during early trunk formation in mouse. Genesis. 2008;46:92-103 pubmed publisher
    ..Our results suggest that Sfrps regulation of the canonical and noncanonical pathways is essential for proper trunk formation. ..

More Information


  1. Jones C, Chen P. Planar cell polarity signaling in vertebrates. Bioessays. 2007;29:120-32 pubmed
    ..This review summarizes the morphogenesis of this model system to assist the interpretation of the emerging data and proposes molecular mechanisms underlying PCP signaling in vertebrates. ..
  2. Torban E, Wang H, Patenaude A, Riccomagno M, Daniels E, Epstein D, et al. Tissue, cellular and sub-cellular localization of the Vangl2 protein during embryonic development: effect of the Lp mutation. Gene Expr Patterns. 2007;7:346-54 pubmed
    ..Lp) mice show a very severe neural tube defect, craniorachischisis, which is caused by mis-sense mutations in the Vangl2 gene...
  3. Wang J, Mark S, Zhang X, Qian D, Yoo S, Radde Gallwitz K, et al. Regulation of polarized extension and planar cell polarity in the cochlea by the vertebrate PCP pathway. Nat Genet. 2005;37:980-5 pubmed
  4. Lu X, Borchers A, Jolicoeur C, Rayburn H, Baker J, Tessier Lavigne M. PTK7/CCK-4 is a novel regulator of planar cell polarity in vertebrates. Nature. 2004;430:93-8 pubmed
    ..stereociliary bundle orientation, and shows genetic interactions with a mutation in the mouse Van Gogh homologue vangl2. We also show that PTK7 is dynamically localized during hair cell polarization, and that the Xenopus homologue of ..
  5. 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...
  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
    ..To begin to elucidate the mechanisms of PCP in vertebrates, the localization of the protein Vangl2 (Van Gogh-like) was determined during the development of the mammalian cochlea...
  7. 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 ..
  8. Qian D, Jones C, Rzadzinska A, Mark S, Zhang X, Steel K, et al. Wnt5a functions in planar cell polarity regulation in mice. Dev Biol. 2007;306:121-33 pubmed
    ..Finally, we show that Wnt5a is required for proper subcellular distribution of a PCP protein, Ltap/Vangl2, and that Wnt5a interacts genetically with Ltap/Vangl2 for uniform orientation of stereocilia, cochlear extension, ..
  9. Merte J, Jensen D, Wright K, Sarsfield S, Wang Y, Schekman R, et al. Sec24b selectively sorts Vangl2 to regulate planar cell polarity during neural tube closure. Nat Cell Biol. 2010;12:41-6; sup pp 1-8 pubmed publisher
    ..b>Vangl2, a key component of the PCP-signalling pathway critical for convergent extension, is selectively sorted into COPII ..
  10. 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
    ..extension in this defect, we vitally labelled the midline of loop-tail (Lp) embryos mutant for the PCP gene Vangl2. Injection of DiI into the node, and electroporation of a GFP expression vector into the midline neural plate, ..
  11. Deans M, Antic D, Suyama K, Scott M, Axelrod J, Goodrich L. Asymmetric distribution of prickle-like 2 reveals an early underlying polarization of vestibular sensory epithelia in the inner ear. J Neurosci. 2007;27:3139-47 pubmed
  12. Wansleeben C, Feitsma H, Montcouquiol M, Kroon C, Cuppen E, Meijlink F. Planar cell polarity defects and defective Vangl2 trafficking in mutants for the COPII gene Sec24b. Development. 2010;137:1067-73 pubmed publisher
    ..isoforms are thought to be directly involved in cargo selection, and we present evidence that Sec24b deficiency specifically affects transport of the PCP core protein Vangl2, based on experiments in embryos and in cultured primary cells.
  13. Montcouquiol M, Rachel R, Lanford P, Copeland N, Jenkins N, Kelley M. Identification of Vangl2 and Scrb1 as planar polarity genes in mammals. Nature. 2003;423:173-7 pubmed
    ..Here we show that a mutation in Vangl2, a mammalian homologue of the Drosophila PCP gene Strabismus/Van Gogh, results in significant disruptions in the ..
  14. Murdoch J, Henderson D, Doudney K, Gaston Massuet C, Phillips H, Paternotte C, et al. Disruption of scribble (Scrb1) causes severe neural tube defects in the circletail mouse. Hum Mol Genet. 2003;12:87-98 pubmed
    ..circletail genetically interacts with the loop-tail mutant, and we reveal overlapping expression of Scrb1 with Vangl2, the gene mutated in loop-tail...
  15. Phillips H, Murdoch J, Chaudhry B, Copp A, Henderson D. Vangl2 acts via RhoA signaling to regulate polarized cell movements during development of the proximal outflow tract. Circ Res. 2005;96:292-9 pubmed
    ..mice develop double outlet right ventricle and aortic arch defects, and the defects are caused by mutations in the Vangl2 gene...
  16. Le Grand F, Jones A, Seale V, Scime A, Rudnicki M. Wnt7a activates the planar cell polarity pathway to drive the symmetric expansion of satellite stem cells. Cell Stem Cell. 2009;4:535-47 pubmed publisher
    ..Wnt7a signaling induced the polarized distribution of the planar cell polarity effector Vangl2. Silencing of Vangl2 inhibited Wnt7a action on satellite stem cell expansion...
  17. Katoh M. Structure and expression of Strabismus 1 gene on human chromosome 1q21-q23. Int J Oncol. 2002;20:1197-203 pubmed
    Xenopus Strabismus (Stbm) is a negative regulator of the WNT - beta-catenin signaling pathway. Strabismus 1 (STB1/VangL2) and Strabismus 2 (STB2/Vangl1) are human homologues of Xenopus Stbm and Drosophila Stbm/ Van Gogh (Vang) STB1 and ..
  18. Jones C, Roper V, Foucher I, Qian D, Banizs B, Petit C, et al. Ciliary proteins link basal body polarization to planar cell polarity regulation. Nat Genet. 2008;40:69-77 pubmed
    ..Thus, our data uncover a distinct requirement for ciliary genes in basal body positioning and morphological polarization during PCP regulation. ..
  19. Dawe C, Kooistra M, Fairbridge N, Pisio A, McDermid H. Role of chromatin remodeling gene Cecr2 in neurulation and inner ear development. Dev Dyn. 2011;240:372-83 pubmed publisher
    ..Although limited genetic interaction for NTDs was seen with Vangl2, a microarray analysis of PCP genes did not reveal a direct connection to this pathway...
  20. Fenstermaker A, Prasad A, Bechara A, Adolfs Y, Tissir F, Goffinet A, et al. Wnt/planar cell polarity signaling controls the anterior-posterior organization of monoaminergic axons in the brainstem. J Neurosci. 2010;30:16053-64 pubmed publisher
    ..We find that 5-HT and mdDA axons express the core planar cell polarity components Frizzled3, Celsr3, and Vangl2. In addition, monoaminergic projections show anterior-posterior guidance defects in Frizzled3, Celsr3, and Vangl2 ..
  21. 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
    ..The membrane distribution of Vangl2 and Fzd3, two key planar cell polarity proteins, was disturbed in Celsr2 mutants, and even more so in Celsr2 and ..
  22. Babayeva S, Zilber Y, Torban E. Planar cell polarity pathway regulates actin rearrangement, cell shape, motility, and nephrin distribution in podocytes. Am J Physiol Renal Physiol. 2011;300:F549-60 pubmed publisher
    ..In reciprocal experiments, siRNA depletion of the core PCP gene Vangl2 reduced the number of cell projections and decreased stress fibers and cell motility...
  23. Hagiwara A, Yasumura M, Hida Y, Inoue E, Ohtsuka T. The planar cell polarity protein Vangl2 bidirectionally regulates dendritic branching in cultured hippocampal neurons. Mol Brain. 2014;7:79 pubmed publisher
    ..Here we explored the finding that a loss of function of Vangl2 results in a significant reduction in spine density and complexity of dendritic branching...
  24. Chen H, Mruk D, Lee W, Cheng C. Planar Cell Polarity (PCP) Protein Vangl2 Regulates Ectoplasmic Specialization Dynamics via Its Effects on Actin Microfilaments in the Testes of Male Rats. Endocrinology. 2016;157:2140-59 pubmed publisher
    ..In adult rat testes, Sertoli and germ cells were found to express PCP core proteins (eg, Van Gogh-like 2 [Vangl2]), effectors, ligands, and signaling proteins...
  25. 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. ..
  26. Yao R, Natsume Y, Noda T. MAGI-3 is involved in the regulation of the JNK signaling pathway as a scaffold protein for frizzled and Ltap. Oncogene. 2004;23:6023-30 pubmed
    ..Furthermore, we also demonstrate that MAGI-3 interacts with Ltap, a mouse homolog of the Drosophila PCP protein, stbm, and that these three molecules can form a ternary complex...
  27. Escobedo N, Contreras O, Muñoz R, Farías M, Carrasco H, Hill C, et al. Syndecan 4 interacts genetically with Vangl2 to regulate neural tube closure and planar cell polarity. Development. 2013;140:3008-17 pubmed publisher
    ..mouse development, we evaluated a possible interaction between a null mutation of Sdc4 and the loop-tail allele of Vangl2. Sdc4 is expressed in multiple tissues, but particularly in the non-neural ectoderm, hindgut and otic vesicles...
  28. May Simera H, Petralia R, Montcouquiol M, Wang Y, Szarama K, Liu Y, et al. Ciliary proteins Bbs8 and Ift20 promote planar cell polarity in the cochlea. Development. 2015;142:555-66 pubmed publisher
    ..deletion of either Bbs8 or Ift20 results in disruptions in asymmetric accumulation of the core PCP molecule Vangl2 in cochlear cells, suggesting a role for Bbs8 and/or Ift20, possibly upstream of core PCP asymmetry...
  29. Nagaoka T, Kishi M. The planar cell polarity protein Vangl2 is involved in postsynaptic compartmentalization. Neurosci Lett. 2016;612:251-255 pubmed publisher
    ..In the present report, we show that the planar cell polarity (PCP) protein Van Gogh-like 2 (Vangl2) plays a role in this regionalization...
  30. Suriben R, Kivimäe S, Fisher D, Moon R, Cheyette B. Posterior malformations in Dact1 mutant mice arise through misregulated Vangl2 at the primitive streak. Nat Genet. 2009;41:977-85 pubmed publisher
    ..Notably, heterozygous mutation of Vangl2, a transmembrane component of the planar cell polarity (PCP) pathway, rescued recessive Dact1 phenotypes, whereas ..
  31. Heydeck W, Liu A. PCP effector proteins inturned and fuzzy play nonredundant roles in the patterning but not convergent extension of mammalian neural tube. Dev Dyn. 2011;240:1938-48 pubmed publisher
    ..We further show that neither gene exhibits obvious genetic interaction with the core PCP regulator Vangl2 in convergent extension or patterning of the neural tube...
  32. Carroll E, Gerrelli D, Gasca S, Berg E, Beier D, Copp A, et al. Cordon-bleu is a conserved gene involved in neural tube formation. Dev Biol. 2003;262:16-31 pubmed
    ..Finally, we show that cobl interacts with the neurulation gene Vangl2 to facilitate midbrain neural tube closure, demonstrating roles for both cobl and Vangl2 in midbrain neurulation.
  33. Nagaoka T, Tabuchi K, Kishi M. PDZ interaction of Vangl2 links PSD-95 and Prickle2 but plays only a limited role in the synaptic localisation of Vangl2. Sci Rep. 2015;5:12916 pubmed publisher
    ..Although the vertebrate regulator of planar cell polarity Van Gogh-like 2 (Vangl2) was recently described as a postsynaptic molecule with a PDZ-binding motif, the role of its PDZ interaction at ..