Transcriptional functionality of germ line p53 mutants influences cancer phenotype.

ABSTRACT:

Purpose

The TP53 tumor suppressor gene encodes a sequence-specific transcription factor that is able to transactivate several sets of genes, the promoters of which include appropriate response elements. Although human cancers frequently contain mutated p53, the alleles as well as the clinical expression are often heterogeneous. Germ line mutations of TP53 result in cancer proneness syndromes known as Li-Fraumeni, Li-Fraumeni--like, and nonsyndromic predisposition with or without family history. p53 mutants can be classified as partial deficiency alleles or severe deficiency alleles depending on their ability to transactivate a set of human target sequences, as measured using a standardized yeast-based assay (see http://www.umd.be:2072/index.html). We have investigated the extent to

SUBMITTER: Monti P 

PROVIDER: S-EPMC2128783 | biostudies-literature | 2007 Jul

REPOSITORIES: biostudies-literature