Type and Frequency of p53 Mutations in Tumors of the Nervous System and Its Coverings

Abstract

The p53 tumor suppressor gene encodes a nuclear phosphoprotein which is involved in the regulation of cell proliferation (Boyd and Barrett 1990). The wild-type p53 gene acts as a tumor suppressor gene, whereas some p53 mutations occurring within highly conserved regions not only cause loss of tumor suppressor function but may activate p53 to an oncogene in a dominant negative fashion (Finlay et al. 1989; Eiyahu et al. 1989). A variety of human tumors have been shown to contain either a loss of both alleles of the p53 gene, a loss of one allele of the p53 gene, and one p53 allele with an associated point mutation, insertion, or deletion of the remaining allele or an inactivation of the p53 gene in one allele but a normal (wild-type) sequence in the other. The rapidly accumulating data on p53 genetic alterations indicate that it may constitute the gene most frequently involved in human oncogenesis (Hollstein et al. 1991; Levine et al. 1991). In this review, we summarize all the available data on p53 mutations in human nervous system tumors.