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Gene Review

Vangl2  -  vang-like 2 (van gogh, Drosophila)

Mus musculus

Synonyms: C530001F03Rik, Loop-tail protein 1, Loop-tail-associated protein, Lp, Lpp1, ...
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Disease relevance of Vangl2

  • These findings suggest a molecular mechanism for the neural tube defect seen in Lp mice [1].
  • The pattern of delayed neuropore closure in Lp/+ embryos resembles that caused by the ct and Sp mutations and is likely to be responsible for the development of tail defects (i.e., looped tails) and spina bifida in Lp/+ mice [2].
  • In this study, we describe complex cardiovascular defects in Lp homozygotes, which include double-outlet right ventricle, with obligatory perimembranous ventricular septal defects, and double-sided aortic arch, with associated abnormalities in the aortic arch arteries [3].

High impact information on Vangl2

  • Polarization defects in animals heterozygous for Vangl2 and Scrb1 are comparable with Vangl2 homozygotes, demonstrating genetic interactions between these genes in the regulation of PCP in mammals [4].
  • Linkage studies indicate that gld is located between Pep-3 and Lp on chromosome 1 [5].
  • This locus, designated Bxv-1, mapped to the same site on chromosome 1 in all strains: Id-1-Pep-3-[Bxv-1-Lp] [6].
  • This review will summarize recent findings documenting the involvement of Stbm (Vangl2) and associated proteins in planar cell polarity, non-canonical Wnt signaling and convergent extension movements [7].
  • 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 [8].

Chemical compound and disease context of Vangl2

  • The effects of retinoic acid (RA) on the manifestation and nature of neural tube defects (NTD) in heterozygous embryos of mutant mice carrying the gene loop-tail (Lp) and in normal (+/+) littermates and embryos from normal homozygous matings were compared with NTD that occur in untreated abnormal homozygous (Lp/Lp) embryos [9].

Biological context of Vangl2


Anatomical context of Vangl2

  • In epithelial cells, MAGI-3, frizzled-4, and Ltap colocalized at cell contact sites, indicating that these molecules form a physiologically significant complex [13].
  • Intriguing, the Loop-tail mutation that disrupts both convergent extension in the neuroepithelium and PCP in the cochlea does not disrupt Dvl2 membrane distribution in the neuroepithelium, in contrast to its drastic effect on Dvl2 localization in the cochlea [10].
  • The mouse looptail (Lp) mutation is an established model for neural tube defects with homozygous Lp embryos showing an open neural tube from the caudal midbrain to the tip of the tail [11].
  • The loop-tail (Lp) mutant mouse provides a model for the most severe NTD, craniorachischisis, in which the brain and spinal cord remain open [8].
  • Heterozygous Lp/+ embryos initiate neural tube closure at the cervical/hindbrain boundary with a slight delay compared with +/+ littermates [2].

Associations of Vangl2 with chemical compounds


Other interactions of Vangl2

  • The Lp gene has been mapped to a 0.6-cM interval on mouse chromosome 1 delineated by two clusters of markers, Fcer1gamma/Usf1/D1Mit113/D1Wsu1 on the proximal side and Fcer1alpha/Spna1/D1Mit149 distally [11].
  • Nhlh1, a member of the basic helix-loop-helix family of transcription factors, is expressed in the developing neural tube in structures affected by the Lp mutation and has been regionally assigned to the distal part of mouse Chr 1 [12].
  • The Lp interval, between D1Mit113 and Tagln2, can be spanned by two nonchimeric overlapping YACs that define a physical distance of approximately 1 Mb [17].
  • Test-cross data show the following gene order and recombination percentages: Dip-1 19.0 +/- 3.8% Lp 7.4 +/- 2.2% Akp-1 [18].
  • 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 [19].

Analytical, diagnostic and therapeutic context of Vangl2


  1. Independent mutations in mouse Vangl2 that cause neural tube defects in looptail mice impair interaction with members of the Dishevelled family. Torban, E., Wang, H.J., Groulx, N., Gros, P. J. Biol. Chem. (2004) [Pubmed]
  2. Developmental basis of severe neural tube defects in the loop-tail (Lp) mutant mouse: use of microsatellite DNA markers to identify embryonic genotype. Copp, A.J., Checiu, I., Henson, J.N. Dev. Biol. (1994) [Pubmed]
  3. Cardiovascular defects associated with abnormalities in midline development in the Loop-tail mouse mutant. Henderson, D.J., Conway, S.J., Greene, N.D., Gerrelli, D., Murdoch, J.N., Anderson, R.H., Copp, A.J. Circ. Res. (2001) [Pubmed]
  4. Identification of Vangl2 and Scrb1 as planar polarity genes in mammals. Montcouquiol, M., Rachel, R.A., Lanford, P.J., Copeland, N.G., Jenkins, N.A., Kelley, M.W. Nature (2003) [Pubmed]
  5. A new mutation, gld, that produces lymphoproliferation and autoimmunity in C3H/HeJ mice. Roths, J.B., Murphy, E.D., Eicher, E.M. J. Exp. Med. (1984) [Pubmed]
  6. Genetic mapping of xenotropic murine leukemia virus-inducing loci in five mouse strains. Kozak, C.A., Rowe, W.P. J. Exp. Med. (1980) [Pubmed]
  7. Van Gogh-like2 (Strabismus) and its role in planar cell polarity and convergent extension in vertebrates. Torban, E., Kor, C., Gros, P. Trends Genet. (2004) [Pubmed]
  8. Severe neural tube defects in the loop-tail mouse result from mutation of Lpp1, a novel gene involved in floor plate specification. Murdoch, J.N., Doudney, K., Paternotte, C., Copp, A.J., Stanier, P. Hum. Mol. Genet. (2001) [Pubmed]
  9. Cranial effects of retinoic acid in the loop-tail (Lp) mutant mouse. Wilson, D.B., Wyatt, D.P., Gookin, J.L. J. Craniofac. Genet. Dev. Biol. (1990) [Pubmed]
  10. Dishevelled genes mediate a conserved mammalian PCP pathway to regulate convergent extension during neurulation. Wang, J., Hamblet, N.S., Mark, S., Dickinson, M.E., Brinkman, B.C., Segil, N., Fraser, S.E., Chen, P., Wallingford, J.B., Wynshaw-Boris, A. Development (2006) [Pubmed]
  11. Physical delineation of a 700-kb region overlapping the Looptail mutation on mouse chromosome 1. Underhill, D.A., Mullick, A., Groulx, N., Beatty, B.G., Gros, P. Genomics (1999) [Pubmed]
  12. Nhlh1, a basic helix-loop-helix transcription factor, is very tightly linked to the mouse looptail (Lp) mutation. Mullick, A., Groulx, N., Trasler, D., Gros, P. Mamm. Genome (1995) [Pubmed]
  13. MAGI-3 is involved in the regulation of the JNK signaling pathway as a scaffold protein for frizzled and Ltap. Yao, R., Natsume, Y., Noda, T. Oncogene (2004) [Pubmed]
  14. Alterations in cranial morphogenesis in the Lp mutant mouse. Wilson, D.B., Wyatt, D.P. J. Craniofac. Genet. Dev. Biol. (1995) [Pubmed]
  15. Vector targeting makes 5-fluorouracil chemotherapy less toxic and more effective in animal models of epithelial neoplasms. Akbulut, H., Tang, Y., Maynard, J., Zhang, L., Pizzorno, G., Deisseroth, A. Clin. Cancer Res. (2004) [Pubmed]
  16. Early development of the brain and spinal cord in dysraphic mice: a transmission electron microscopic study. Wilson, D.B., Finta, L.A. J. Comp. Neurol. (1980) [Pubmed]
  17. Physical and transcriptional map of a 3-Mb region of mouse chromosome 1 containing the gene for the neural tube defect mutant loop-tail (Lp). Eddleston, J., Murdoch, J.N., Copp, A.J., Stanier, P. Genomics (1999) [Pubmed]
  18. Genetic variation in alkaline phosphatase of the house mouse (Mus musculus) with emphasis on a manganese-requiring isozyme. Wilcox, F.H., Hirschhorn, L., Taylor, B.A., Womack, J.E., Roderick, T.H. Biochem. Genet. (1979) [Pubmed]
  19. Asymmetric localization of Vangl2 and Fz3 indicate novel mechanisms for planar cell polarity in mammals. Montcouquiol, M., Sans, N., Huss, D., Kach, J., Dickman, J.D., Forge, A., Rachel, R.A., Copeland, N.G., Jenkins, N.A., Bogani, D., Murdoch, J., Warchol, M.E., Wenthold, R.J., Kelley, M.W. J. Neurosci. (2006) [Pubmed]
  20. Strabismus, a novel gene that regulates tissue polarity and cell fate decisions in Drosophila. Wolff, T., Rubin, G.M. Development (1998) [Pubmed]
  21. Ultrastructural analysis of the midaxial extracellular matrix in spinal dysraphism. Wilson, D.B., Wyatt, D.P. Virchows Arch., B, Cell Pathol. (1991) [Pubmed]
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