Phenotypes Associated with This Genotype

Genotype
MGI:8375452

• Allelic Composition: Men1^tm1.2Zqw/Men1^tm1.2Zqw; Tg(Runx2-icre)1Jtuc/0
• Genetic Background: involves: 129P2/OlaHsd * FVB/N

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

neoplasm

increased fibroma incidence ( J:257886 )

• mice exhibit ossifying fibroma in their mandibles at 15 months of age

• some mice exhibit lesions in maxilla

• tumors consist of dense proliferations of monomorphic spindle cells surrounding abundant osteoid and cement-like matrix deposits at 3 and 6 months of age

• onset of ossifying fibroma development occurs as focal spindle cell proliferations at the periodontal ligament around the molar roots at 3 months of age, however fibrous hyperplasia is not evident at periodontal ligament around the incisors

• tumor volume progressively increases from 6 months to 12 months and as tumor volume increases, the alveolar bone volume of the affected mandible is reduced, trabecular thickness and trabecular number are decreased and trabecular separation is increased

• no other one lesions are detected in the remaining appendicular or axial skeleton

• ossifying fibroma-derived mesenchymal stem cells (OFMSCs) form more single colonies and proliferate faster

skeleton

arrested osteoblast differentiation ( J:257886 )

• lesional stromal spindle cells isolated from ossifying fibroma are arrested at an early osteoblast differentiation stage

cellular

arrested osteoblast differentiation ( J:257886 )

• lesional stromal spindle cells isolated from ossifying fibroma are arrested at an early osteoblast differentiation stage

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
ossifying fibroma		DOID:180		J:257886