The Li-Fraumeni syndrome is a rare autosomal-dominant disease whose ha
llmark is a predisposition to a wide range of cancers among members of
a family, Many of these families have a germline mutation within the
tumor suppressor gene TP53, which encodes the p53 protein. The inherit
ance of a mutant TP53 allele results in a 25-fold increase in the chan
ce of developing cancer by 50 years of age, compared with the general
population, TP53 mutations are also very common in the development of
somatic tumors, This article reviews the biological and biochemical ro
le of p53 in the susceptibility to cancer in Li-Fraumeni syndrome.