| First Author | Else T | Year | 2009 |
| Journal | Cancer Cell | Volume | 15 |
| Issue | 6 | Pages | 465-76 |
| PubMed ID | 19477426 | Mgi Jnum | J:149664 |
| Mgi Id | MGI:3848842 | Doi | 10.1016/j.ccr.2009.04.011 |
| Citation | Else T, et al. (2009) Genetic p53 deficiency partially rescues the adrenocortical dysplasia phenotype at the expense of increased tumorigenesis. Cancer Cell 15(6):465-76 |
| abstractText | Telomere dysfunction and shortening induce chromosomal instability and tumorigenesis. In this study, we analyze the adrenocortical dysplasia (acd) mouse, harboring a mutation in Tpp1/Acd. Additional loss of p53 dramatically rescues the acd phenotype in an organ-specific manner, including skin hyperpigmentation and adrenal morphology, but not germ cell atrophy. Survival to weaning age is significantly increased in Acd(acd/acd) p53(-/-) mice. On the contrary, p53(-/-) and p53(+/-) mice with the Acd(acd/acd) genotype show a decreased tumor-free survival, compared with Acd(+/+) mice. Tumors from Acd(acd/acd) p53(+/-) mice show a striking switch from the classic spectrum of p53(-/-) mice toward carcinomas. The acd mouse model provides further support for an in vivo role of telomere deprotection in tumorigenesis. |
