The complexity of Wnt intracellular signaling pathways parallels the complexity observed in the diversity of Wnt receptors. Wnt receptors include the Frizzled family, Low Density Lipoprotein Receptor-related Proteins-5/6 (LRP-5/6), Receptor Tyrosine Kinase-like Orphan Receptor-1/2 (ROR1/2), and related to tyrosine (Y) kinase (Ryk).

Frizzled receptors are seven transmembrane G protein-coupled receptors for the Wnt family of glycoproteins. This receptor family contains at least ten different members that mediate distinct, tissue-specific effects. Structurally, all members of the Frizzled family are similar. Each contains a divergent N-terminal signal peptide, a highly conserved extracellular cysteine-rich domain (CRD), a variable length linker region, a seven-pass transmembrane domain, and a variable length C-terminal end. Wnt ligands bind to Frizzled receptors through the CRD, and may also require a co-receptor, such as LRP-5, LRP-6, ROR, or Ryk, to activate downstream signaling pathways. LRP-5 and LRP-6 are orthologs of the Drosophila receptor Arrow, and are associated with the canonical beta-Catenin-dependent signaling pathway. ROR1/2 and Ryk are additional Wnt co-receptors that are associated with non-canonical Wnt signaling pathways. Since Wnt ligands have different affinities for different Frizzled receptors, activation of a given Wnt/Frizzled signaling cascade is dependent on the Wnt ligand and the cellular context of the interaction. Wnt signaling is involved in a variety of developmental processes including cell fate determination, cell polarity, tissue patterning, and control of cell proliferation. In addition, it plays a role in the maintenance of adult tissue homeostasis.

Flamingo Subfamily

CELSR2CELSR3

Wnt Signaling Modulators

Given that Wnt signaling underlies a diverse range of complex biological functions, modulators of Wnt signaling are of great interest. A number of proteins can either enhance or inhibit Wnt signaling, including Glypicans, R-Spondin proteins, Kremen-1, Kremen-2, Norrin, Sclerostin (SOST), and MESDC2. Glypicans are a family of six heparan sulfate proteoglycans that are anchored to the plasma membrane by a glycosylphosphatidylinositol linkage. These proteins enhance Wnt signaling by stabilizing the interaction between Wnt proteins and Frizzled receptors. Members of the R-Spondin family of proteins (Rspo1-4) also function as positive regulators of Wnt/beta-Catenin-dependent signaling by interfering with Dkk-1-mediated internalization of the Wnt co-receptor, LRP-6. In contrast, Kremen-1 and Kremen-2 antagonize Wnt signaling by forming a complex with Dkk proteins and LRP-5/6 that stimulates LRP-5/6 internalization. MESDC2 and SOST also regulate Wnt signaling through LRP-5/6. MESDC2 is required for proper folding and expression of LRP-5/6, while SOST binds LRP-5/6 and inhibits its ability to function as a co-receptor. Other proteins may regulate Wnt signaling by directly binding to Wnt ligands or Wnt receptors. For example, Norrin is a secreted protein associated with the extracellular matrix that is not related to the Wnt family, but binds to Frizzled-4/LRP and activates the canonical Wnt signaling pathway.