Table 1.
Mouse models exhibiting p53-dependent brain developmental phenotypes.
| Mouse Models | p53-Dependent Brain Developmental Phenotypes | p53 Status |
|---|---|---|
| p53−/− [13,14] | Female-specific exencephaly, spina bifida, retinal dysplasia | p53 deletion |
| p53N236S/N236S [22] | Female specific exencephaly and spina bifida | p53 missense mutation |
| p53−/−; Bim+/− [26] | 100% penetrate, female-exclusive exencephaly | p53 deletion |
| Pax3Sp/Sp [33] | Exencephaly | p53 stabilization |
| Mdm2FM/+; Nestin-Cre [35] | Hydranencephaly | p53 stabilization |
| Mdm4FX/+; Nestin-Cre [35] | Porencephaly | p53 stabilization |
| Nbnflox/flox; Nestin-Cre [36] | Microcephaly | p53 stabilization |
| Nde1−/− [37] | Microcephaly | p53 stabilization |
| Cep63T/T [38] | Microcephaly | p53 stabilization |
|
Tubb5E401K/E401K; Nestin-Cre [39] Tubb5flox/+; Nestin-Cre [39] |
Microcephaly | p53 stabilization |
| Eif4a3flox/+; Emx1-Cre [40] | Microcephaly | p53 stabilization |
| Rbm8aflox/+; Emx1-Cre [40] | Microcephaly | p53 stabilization |
| Magohflox/+; Emx1-Cre [40] | Microcephaly | p53 stabilization |
| CitK−/− [41] | Microcephaly | p53 stabilization |
| Kif20bm/m [42] | Microcephaly | p53 stabilization |
|
AspmSA/SA [43] Aspmflox/flox; Math1-Cre [43] |
Microcephaly (hypoplastic cerebellum) | p53 stabilization |
| p53515C/515C; Mdm2−/− [44] | Cerebellar defects | Mutant p53 stabilization |
Note: p53-dependent brain developmental phenotypes in these models are caused by p53 deletion, mutation or p53 protein stabilization. Some mouse models may also exhibit non-CNS phenotypes that are not described here.