Table 1.
Core PCP factors and their molecular features
| Drosophila gene | Mouse gene(s) | Molecular features and function(s) | Primary PCP Refs |
|---|---|---|---|
| Fz/Fmi core group | |||
| frizzled (fz) | Fz3, Fz6 | 7-pass transmembrane receptors; bind Wnt ligands and also activate Wnt–β-catenin signaling; interact with Dsh–Dvl proteins; recruit Dsh and Dgo to the plasma membrane; can co-IP with Fmi | [19,20,40,47,63] |
| disheveled (dsh) | Dishevelled-like 1–3 (Dvl1, Dvl2, Dvl3) | Cytoplasmic protein containing DIX, PDZ and DEP domains; recruited to membrane by Fz; binds to Fz, Pk, and Dgo (all species tested) and has also been shown to bind to Vang–Stbm in Xenopus | [64–70] |
| Van Gogh (Vang)/strabismus (stbm) | Vang-like 1 and 2 (Vangl1, Vangl2), Vangl2 also known as Looptail zebrafish mutant: trilobite (tri) | Novel four-pass transmembrane proteins; bind Pk, and Dgo (and Dsh in Xenopus); recruit Pk to the membrane; can co-IP with Fmi–Celsr | [18,21,30,41,53, 70–73] |
| prickle (pk) (also known as prickle-spiny legs) | Pk1 and Pk2 | Cytoplasmic protein with 3 LIM domains and a PET domain; recruited to the plasma membrane by Vang; can interact directly with Dsh, Vang and Dgo; competes with Dgo for Dsh binding. | [30,31,53,69, 74–76] |
| flamingo (fmi)/starry night (stan) |
Celsr1, Celsr2 and Celsr3 (Cadherin-EGF-LAG 7-pass G-type receptor 1) Zebrafish mutant: off-road (Celsr2) |
Multiple Cadherin repeats in extracellular domain, seven-pass transmembrane receptor features; capable of homophilic cell adhesion Can co-IP with Fz and Vang |
[18,33,47,77,78] |
| diego (dgo) | diversin (also known as ankyrin repeat domain 6; ankrd6); inversin (invs) | Cytoplasmic proteins containing 6 ankyrin repeats; recruited to membrane by Fz; bind Dsh, Vang and Pk; compete with Pk for Dsh binding | [62,69,79–81] |
| Fat/Ds core group | |||
| fat (ft) | Fat1, Fat2, Fat3, Fat4 (Fat-j) | Proto-cadherins with single pass transmembrane; binds Ds extracellularly (Drosophila); interacts with Atrophin in cytosol | [52,82–84,85] |
| dachsous (ds) | Dchs1, Dchs2 (more related genes possible) | Proto-cadherin with single pass transmembrane; binds Ft extracellularly | [52,82,83,86,87] |
| four-jointed (fj) | Fjx1 | Type II membrane protein; localized in Golgi; kinase, can phosphorylate Ft, Ds | [52,82,83,88,89] |
| approximated (app) | human DHHC9, DHHC14, DHHC18 | DHHC palmitoyltransferase; negative regulator of Ft | [90] |
| atrophin (atro),also known as Grunge (Gug) | Atrophin (human) | Transcriptional co-repressor; can bind to Ft cytoplamsic tail | [91,92] |