Fig. 10: Compound mutant phenotypes of Vangl2, Grhl3, Usp39, and Ube1 genes during mouse development and schematic model of USP39/GRHL3 complex in the de-ubiquitination pathway.

a Schematic illustrations of phenotypes associated with non-canonical Wnt signaling during mouse development: solid red crosses denote defective formation with expression of complete penetrance (100%). Open red crosses denote defective formation but the penetrance is lower or the phenotype is milder. Solid arrows indicate enhancement of the penetrance or severity of defective phenotypes observed by removal of one copy of the Usp39 gene. The open arrow indicates restoration of the severity of the defect in Usp39−/− embryos by removal of one copy of the Ube1 gene. b A schematic model representing how the GRHL3/USP39 interaction can stabilize the PRICKLE1 protein as a target of PCP-related molecules via deubiquitination. ND not determined, PCP planar cell polarity.