This article reviews the recent molecular and clinical studies that charact
erize the role of p53 in pathologies of the central nervous system, p53 has
many important biological functions, notably, maintenance of DNA stability
and regulation of apoptosis. These features are essential to avoid cellula
r transformation and ensure normal brain development. Lack of p53 function
in the brain results in tumor formation in the astrocytic and lymphoid line
ages and in severe neurodevelopmental diseases, such as exencephaly.