Phenotypes Associated with This Genotype

Genotype
MGI:5796169

• Allelic Composition: Prkar1a^tm1.2Lsk/Prkar1a⁺; Tg(Col1a1-cre)1Kry/0; Trp53^tm1Brn/Trp53^tm1Brn
• Genetic Background: involves: 129P2/OlaHsd * 129S1/Sv * 129X1/SvJ * FVB/N

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

mortality/aging

premature death ( J:234128 )

• mice survive to around 17 weeks of age

neoplasm

increased osteosarcoma incidence ( J:234128 )

• mice develop osteosarcoma within 17 weeks of age

• multiple tumors among long bones, spine, jaw, and skull

• tumors are composed of malignant-appearing osteoblasts with woven bone as the predominant matrix and occasional chondroblastic or fibroblastic phenotype

• tumors are marked by high TNFSF11 (RANKL)/low TNFRSF11A (RANK) levels

skeleton

increased osteosarcoma incidence ( J:234128 )

• mice develop osteosarcoma within 17 weeks of age

• multiple tumors among long bones, spine, jaw, and skull

• tumors are composed of malignant-appearing osteoblasts with woven bone as the predominant matrix and occasional chondroblastic or fibroblastic phenotype

• tumors are marked by high TNFSF11 (RANKL)/low TNFRSF11A (RANK) levels

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
osteosarcoma		DOID:3347	OMIM:259500	J:234128