Abnormalities of the p53 Tumour Suppressor Gene in Mature B-cell Neoplasms
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The p53 gene, located on the short arm of human chromosome 17 at 17p13, codes for a 393 aminoacid phosphoprotein, which acts as a transcription factor and is involved in the control of many different cellular processes. It is the most frequently mutated gene in neoplasia and mutations have been observed in 231 of the 393 codons, including all but one codon of the DNA binding domain. p53 abnormalities in mature B-cell lymphoproliferative disorders (B-LPDs) occur in up to 75% of cases and are mostly detected in patients with advanced clinical stages. B-LPDs encompass a heterogeneous group of clinically important lymphoid malignancies with a complex biology, varying natural history and prognosis that makes their classification and treatment difficult. Despite many publications concerning the role of p53 abnormalities in the development of B-LPDs and the prognostic implications of detecting aberrant p53 function, it is difficult to draw firm conclusions as studies have varied with respect to patient selection and classification and techniques used. This review focuses on the available data pertaining to p53 abnormalities in the different mature B-cell neoplasms and summarises the incidences of abnormalities, the mutation patterns encountered and their clinical significance.
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Hematology, 2001, 6 pp. 241 - 254
Taylor & Francis