Phenotypes Associated with This Genotype

Genotype
MGI:5796167

• Allelic Composition: Prkar1a^tm1.2Lsk/Prkar1a⁺; Rb1^tm2Brn/Rb1^tm2Brn; Trp53^tm1Brn/Trp53^tm1Brn; Tg(Col1a1-cre)1Kry/0
• Genetic Background: involves: 129 * 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 do not live longer than 11 weeks

• treatment of mice with RANK-Fc prolongs survival to around to around 13.3 weeks

neoplasm

increased osteosarcoma incidence ( J:234128 )

• mice develop osteosarcoma within 6.3 weeks of age

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

• treatment of mice with RANK-Fc starting at 2.4 weeks of age suppresses tumor growth

• mice develop tumors 4.3 weeks after the discontinuation of RANK-Fc treatment and large numbers of osteoclasts are seen lining the boundary of tumor cells and adjacent osteopetrotic bone

skeleton

increased osteosarcoma incidence ( J:234128 )

• mice develop osteosarcoma within 6.3 weeks of age

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

• treatment of mice with RANK-Fc starting at 2.4 weeks of age suppresses tumor growth

• mice develop tumors 4.3 weeks after the discontinuation of RANK-Fc treatment and large numbers of osteoclasts are seen lining the boundary of tumor cells and adjacent osteopetrotic bone

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