Defects in neural tube formation are among the most common malformatio
ns leading to infant mortality. Although numerous genetic loci appear
to contribute to the defects observed in humans and in animal model sy
stems, few of the genes involved have been characterized at the molecu
lar level, Mice lacking the p53 tumour suppressor gene are predisposed
to tumours, but the viability of these animals indicates that p53 fun
ction is not essential for embryonic development. Here, we demonstrate
that a fraction of p53-deficient embryos in fact do not develop norma
lly. These animals display defects in neural tube closure resulting in
an overgrowth of neural tissue in the region of the midbrain, a condi
tion known as exencephaly.