Notably, Pax3 mutants (Pax3Sp2H, Pax3Sp) are among the very few models in which NTDs have been found to be both preventable by supplemental FA and exacerbated by maternal folate deficiency, imposed by diet and administration of antibiotic to remove folate-synthesizing gut bacteria (Burren et al., 2008; Fleming and Copp, 1998; Wlodarczyk et al., 2006). This evidence concerns the gene PAX3 and folate deficiency.