id: PW:0000200
rgd_url: https://rgd.mcw.edu/rgdweb/pathway/pathwayRecord.html?processType=view&species=Rat&acc_id=PW:0000200
title: wnt signaling, the planar cell polarity pathway
description_xrefs:
- GO:0060071
- KEGG:map04310
description: The planar cell polarity Wnt signaling pathway (PCP) plays a major role in embryonic tissue
  patterning, cell polarization, migration and morphogenesis. The PCP pathway, along with the Wnt-calcium
  pathway, belongs to the non-canonical Wnt signaling in that is independent of beta-catenin, the core
  component of the canonical Wnt pathway. The PCP pathway is less well understood and characterized but
  evidence is rapidly accumulating establishing the core components and the role they play, the commondenominators
  and the distinctions between Drosophila and vertebrates. Frizzled receptors (Fzd) and the intracellular
  dishevelled (Dvl), the latter representing the branching point between canonical and non-canonical pathways,
  are necessary components. A requirement for Wnt ligands is controversial but some, like Wnt5, Wnt7 and
  Wnt11 appear to play a role, at least in vertebrates. Other core components include the membrane proteins
  Vangl2 (Strabismus/Van Gogh homolog) and Celsr1 (Flamingo homolog) and the cytoplasmic Prickle. Additional
  members of the vertebrate PCP pathway include Ptk7 and Scrib and the downstream effector Daam1. The
  asymmetric and polarized membrane association of PCP components has been established in Drosophila;
  a similar, yet distinct pattern has been observed in mammals. Scrib interacting with Vangl2 plays a
  role in the asymmetric positioning of Vangl2 (interaction mediated by the PDZ domains of the two proteins,
  a domain also shared by the modular dishevelled); Celsr1 also contributes to the asymmetric localization
  of Vangl2. Ptk7 also appears to interact with Vangl2 but this interaction plays, remains to be established.
  Vangl can recruit Prickle which then can bind to and antagonize Dvl recruitment by Fzd. Activation of
  Fzd and recruitment of Dvl leads to activation of the members of the Rho family of small monomeric G
  proteins RhoA ¿ via interaction with Daam1, and Rac and subsequent triggering of Rho/Rac/Cdc42 and C-Jun
  N-terminal kinase (JNK) pathways. The former pathway controls cytoskeletal rearrangements and organization
  and the latter impacts on gene expression. Deregulation of the pathway has been implicated in a number
  of human diseases including cancer.
diagram_image: https://rgd.mcw.edu/pathway/PW0000200/Wnt%20signaling%2C%20the%20planar%20cell%20polarity%20pathway/pwmap.png
pathway_studio_file: https://rgd.mcw.edu/pathway/PW0000200/Wnt%20signaling,%20the%20planar%20cell%20polarity%20pathway/Wnt%20signaling,%20the%20planar%20cell%20polarity%20pathway.gpp
diagram_pathway_links:
- id: PW:0000105
  label: Rho/Rac/Cdc42 mediated signaling pathway
- id: PW:0000313
  label: c-Jun N-terminal kinases MAPK signaling pathway
diagram_gene_links:
- id: RGD:1560078
  label: Celsr1
- id: RGD:1305119
  label: Daam1
- id: RGD:735090
  label: Prickle1
- id: RGD:1306723
  label: Prickle2
- id: RGD:1310472
  label: Ptk7
- id: RGD:619755
  label: Rac1
- id: RGD:619921
  label: Rhoa
- id: RGD:1565055
  label: Scrib
- id: RGD:1309442
  label: Vangl2
diagram_chemicals: []
diagram_edges:
- source: Daam1
  relation: associated_with
  target: Dvls
- source: Daam1
  relation: associated_with
  target: Rhoa
- source: Dvls
  relation: associated_with
  target: Fzd_receptors
- source: Dvls
  relation: associated_with
  target: Prickle1
- source: Dvls
  relation: associated_with
  target: Rac1
- source: Fzd_receptors
  relation: associated_with
  target: Wnt_ligands
- source: Prickle1
  relation: associated_with
  target: Vangl2
- source: Prickle1
  relation: negatively_affects
  target: Dvls
- source: Prickle2
  relation: associated_with
  target: Vangl2
- source: Ptk7
  relation: associated_with
  target: Vangl2
- source: Rac1
  relation: positively_affects
  target: Rho/Rac/Cdc42 mediated signaling pathway
- source: Rac1
  relation: positively_affects
  target: c-Jun N-terminal kinases MAPK signaling pathway
- source: Rhoa
  relation: positively_affects
  target: Rho/Rac/Cdc42 mediated signaling pathway
- source: Scrib
  relation: associated_with
  target: Vangl2
diagram_labels:
- Celsr1 --+> Vangl2
- Scrib --+> Vangl2
diagram_other_links:
- href: /wg/home/pathway2/molecular-pathways2/dvl-family
  label: Dvls
- href: /wg/home/pathway2/molecular-pathways2/fzd-family
  label: Fzd_receptors
- href: /wg/home/pathway2/molecular-pathways2/wnt-family
  label: Wnt_ligands
references:
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- id: RGD:2313743
  citation: van Amerongen R, etal., Sci Signal. 2008 Sep 2;1(35):re9.
- id: RGD:5129774
  citation: Veeman MT, etal., Dev Cell. 2003 Sep;5(3):367-77.
- id: RGD:5129825
  citation: Wallingford JB and Habas R, Development. 2005 Oct;132(20):4421-36.
- id: RGD:5129826
  citation: Klein TJ and Mlodzik M, Annu Rev Cell Dev Biol. 2005;21:155-76.
- id: RGD:5129866
  citation: Barrow JR Semin Cell Dev Biol. 2006 Apr;17(2):185-93. Epub 2006 Apr 18.
- id: RGD:5129867
  citation: Montcouquiol M, etal., Annu Rev Neurosci. 2006;29:363-86.
- id: RGD:5129907
  citation: Seifert JR and Mlodzik M, Nat Rev Genet. 2007 Feb;8(2):126-38.
- id: RGD:5129928
  citation: Brynczka C, etal., BMC Genomics. 2007 May 31;8:139.