Phenotypes Associated with This Genotype

Genotype
MGI:3616343

• Allelic Composition: Stk11^tm1.1Mlfr/Stk11⁺; Trp53^tm1Tyj/Trp53⁺
• Genetic Background: involves: 129S2/SvPas * 129S7/SvEvBrd * C57BL/6J

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

mortality/aging

premature death ( J:104347 )

• Approximately 50% die by 10 months of age, significantly sooner than either single heterozygote

digestive/alimentary system

gastric polyps ( J:104347 )

• time to onset of polyposis is similar to single heterozygotes

• 92% of moribund mice between 7 and 14 months of age have gastrointestinal polyps

• polyps are not found before 5.5 months of age, but at 6.5 months are seen in 60% of mice

growth/size/body

distended abdomen ( J:104347 )

• mice have enlarged abdomens due to presence of polyps in pylorus and duodenum

neoplasm

increased hamartoma incidence ( J:104347 )

• polyps are hamartomas

• tumor-free survival rate is about 11 months compared with 17 months for Trp53 single heterozygotes

• 23 of 38 (61%) of double heterozygotes developed a total of 30 tumors

increased lymphoma incidence ( J:104347 )

• 20% (6 of 30) tumors are lymphomas

increased sarcoma incidence ( J:104347 )

• 10% (3 of 30) of the tumors are sarcomas

increased osteosarcoma incidence ( J:104347 )

• 43% of the tumors (13 of the 30) are osteosarcomas

skeleton

increased osteosarcoma incidence ( J:104347 )

• 43% of the tumors (13 of the 30) are osteosarcomas

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Peutz-Jeghers syndrome		DOID:3852	OMIM:175200	J:104347