Phenotypes Associated with This Genotype

Genotype
MGI:5796038

cn23

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

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

Bone tumorigenesis nd abundant osteoclasts in Prkar1a^tm1.2Lsk/Prkar1a⁺ Tg(Col1a1-cre)1Kry/0 mice

neoplasm

increased skeletal tumor incidence ( J:165282 )

• mice develop bone tumors starting at 10 weeks of age, with most mice having tumors by 40 weeks of age

• lesions grow slowly, with 25% of mice having 8-mm tumors by 50 weeks of age

• mice do not develop metastases

• lesions are not typical osteosarcomas as they are not composed of malignant osteoblasts depositing osteoid, but consist of an enlarged marrow space filled with abundant myxoid matrix, containing small fibroblast-like cells surrounded by trabecular-like bone, lined by normal osteoblasts and regions with abundant osteoclasts

skeleton

increased skeletal tumor incidence ( J:165282 )

• mice develop bone tumors starting at 10 weeks of age, with most mice having tumors by 40 weeks of age

• lesions grow slowly, with 25% of mice having 8-mm tumors by 50 weeks of age

• mice do not develop metastases

• lesions are not typical osteosarcomas as they are not composed of malignant osteoblasts depositing osteoid, but consist of an enlarged marrow space filled with abundant myxoid matrix, containing small fibroblast-like cells surrounded by trabecular-like bone, lined by normal osteoblasts and regions with abundant osteoclasts

abnormal bone remodeling ( J:165282 )

• bone within and around lesions is normal and contains osteocytes but is undergoing extensive remodeling