The molecular mechanisms underlying the female bias of neural tube defects are currently unclear. Delbridge et al. present evidence that p53 is required for normal Xist expression and X chromosome inactivation and show in two models that partial failure of X chromosome inactivation is associated with female-biased neural tube defects.
Delbridge, A. R. D., Kueh, A. J., Ke, F., Zamudio, N. M., El-Saafin, F., Jansz, N., … Voss, A. K. (2019). Loss of p53 Causes Stochastic Aberrant X-Chromosome Inactivation and Female-Specific Neural Tube Defects. Cell Reports, 27(2), 442-454.e5. https://doi.org/10.1016/j.celrep.2019.03.048