Table 1.

Mouse mutants of key PCP Genes in a digenic fashion and NTD phenotypes.

Gene and mutant	NTD Type	Penetrance	References
Vangl2Lp/+, Dvl2−/−	CRN	100%	Wang et al. (2006) [25]
Vangl2Lp/+, Dvl3+/−	CRN, EX	30%	Etheridge et al. (2008) [26]
Vangl2Lp/+,ScribCrc/+	CRN or SB	55% CRN, 5% SB	Murdoch et al. (2001) [27]
Vangl2Lp/+,ScribCrc/+	CRN	54%	Murdoch et al. (2014) [2]
Vangl2Lp/+,Celsr1Crsh/+	CRN	not stated	Copp et al. (2003) [41]
Vangl2Lp/+,Celsr1Crsh/+	CRN	54%	Murdoch et al. (2014) [2]
Vangl2Lp/+, Ptk7+/−	SB	95%	Lu et al. (2004) [47]
Vangl2Lp/+,Vangl1+/−	CRN	60%	Torban et al. (2008) [28]
Dvl2−/−,Dvl3+/−	CRN	~100%	Etheridge et al. (2008) [26]
Dvl2+/−,Dvl3−/−	CRN	some	Etheridge et al. (2008) [26]
ScribCrc/+,Celsr1Crsh/+	CRN	8%	Murdoch et al. (2014) [2]

PCP, planar cell polarity; NTD, neural tube defect; CRN, craniorachischisis; SB, spina bifida; EX, exencephaly.