mortality/aging
• mean life span is 15.8 months
|
neoplasm
• compared to Trp53tm1Tyj
|
• 5 of 36 develop B cell lymphomas generally arising in the spleen or mesenteric lymph nodes
|
• the incidence of carcinomas is increased compared to Trp53tm1Tyj heterozygotes
• 5 of 36 develop squamous cell carcinomas and a single case each of transitional cell renal carcinoma and intestinal carcinoma are seen
|
• 2 of 36 develop hepatocellular carcinomas
|
• 7 of 36 develop lung adenocarcinomas, several with malignant features commonly seen in human lung adenocarcinoma including nuclear atypia (5 of 7), desmoplasia (4 of 7), and metaplasias (2 of 7)
|
• 5 of 36 develop squamous cell carcinomas
|
cellular
• resistance to gamma-irradiation induced apoptosis is increased compared to Trp53tm1Tyj hterozygotes
|
• a larger fraction of MEFs are in S phase compared to Trp53tm1Tyj hterozygotes; however DNA damage-induced G1 arrest is intact
|
respiratory system
• 7 of 36 develop lung adenocarcinomas, several with malignant features commonly seen in human lung adenocarcinoma including nuclear atypia (5 of 7), desmoplasia (4 of 7), and metaplasias (2 of 7)
|
liver/biliary system
• 2 of 36 develop hepatocellular carcinomas
|
Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
Li-Fraumeni syndrome | DOID:3012 |
OMIM:PS151623 |
J:95316 |