Mouse Genome Informatics
involves: 129S4/SvJae
phenotype observed in females
phenotype observed in males
N normal phenotype
• mean life span is 15.8 months

• 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
• 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)
• 2 of 36 develop hepatocellular carcinomas
• 5 of 36 develop squamous cell carcinomas

• 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

Mouse Models of Human Disease
Li-Fraumeni Syndrome 1; LFS1 151623 J:95316