Europe PMC

This website requires cookies, and the limited processing of your personal data in order to function. By using the site you are agreeing to this as outlined in our privacy notice and cookie policy.

Mutant p53 gain of function in two mouse models of Li-Fraumeni syndrome.

Author information

Affiliations

  • 1. Center for Cancer Research, Massachusetts Institute of Technology, Cambridge, MA 02139, USA.
      Authors
    • Olive KP 1
    (1 author)

ORCIDs linked to this article

Cell, 01 Dec 2004, 119(6):847-860
https://doi.org/10.1016/j.cell.2004.11.004 PMID: 15607980 

Abstract 

The p53 tumor suppressor gene is commonly altered in human tumors, predominantly through missense mutations that result in accumulation of mutant p53 protein. These mutations may confer dominant-negative or gain-of-function properties to p53. To ascertain the physiological effects of p53 point mutation, the structural mutant p53R172H and the contact mutant p53R270H (codons 175 and 273 in humans) were engineered into the endogenous p53 locus in mice. p53R270H/+ and p53R172H/+ mice are models of Li-Fraumeni Syndrome; they developed allele-specific tumor spectra distinct from p53+/- mice. In addition, p53R270H/- and p53R172H/- mice developed novel tumors compared to p53-/- mice, including a variety of carcinomas and more frequent endothelial tumors. Dominant effects that varied by allele and function were observed in primary cells derived from p53R270H/+ and p53R172H/+ mice. These results demonstrate that point mutant p53 alleles expressed under physiological control have enhanced oncogenic potential beyond the simple loss of p53 function.

Full text links 

Read article at publisher's site: https://doi.org/10.1016/j.cell.2004.11.004

Read article for free, from open access legal sources, via Unpaywall: http://www.cell.com/article/S0092867404010463/pdfUnpaywall

References 

Articles referenced by this article (56)

Show 10 more references (10 of 56)

Citations & impact 

Impact metrics

867
Citations
Jump to Citations
8
Data citations
Jump to Data

Citations of article over time

Alternative metrics

Altmetric item for https://www.altmetric.com/details/5508646
Altmetric
Discover the attention surrounding your research
https://www.altmetric.com/details/5508646

Article citations

Go to all (867) article citations

Data 

Similar Articles 

To arrive at the top five similar articles we use a word-weighted algorithm to compare words from the Title and Abstract of each citation.