Mouse Genome Informatics
cn
    Amer1tm1.1Nbar/Y
Tg(Prrx1-cre)1Cjt/0

involves: 129S4/SvJae * C57BL/6 * SJL
Key:
phenotype observed in females
phenotype observed in males
N normal phenotype
skeleton
• recapitulates appendicular skeletal phenotypes seen in germline null mice
• sharp reduction in the adipocyte content of long bones
• asymmetrical distribution of ossification centers
• increase in the proportion of early osteoblastic cells in primary bone marrow derived mesenchymal progenitor cell cultures compared to wild-type cultures indicating enhanced early osteogenic commitment
• decrease in the late differentiation of osteoblasts in primary bone marrow derived mesenchymal progenitor cell cultures
• primary bone marrow derived mesenchymal progenitor cell cultures continue to produce osteogenic cells under adipogenic conditions
• accumulation of cortical bone in adults
• increase in the osteoblast number relative to surface area
• accumulation of trabecular bone in adults
• increased bone formation rate per tissue volume
• increase in bone mineralized surface per bone surface
• increase in osteoid volume per bone volume indicates a delay in bone mineralization

adipose tissue
• sharp reduction in the adipocyte content of long bones
• severely compromised formation of oil red O-positive adipocytes in primary bone marrow derived mesenchymal progenitor cell cultures

limbs/digits/tail

cellular
• severely compromised formation of oil red O-positive adipocytes in primary bone marrow derived mesenchymal progenitor cell cultures
• increase in the proportion of early osteoblastic cells in primary bone marrow derived mesenchymal progenitor cell cultures compared to wild-type cultures indicating enhanced early osteogenic commitment
• decrease in the late differentiation of osteoblasts in primary bone marrow derived mesenchymal progenitor cell cultures
• primary bone marrow derived mesenchymal progenitor cell cultures continue to produce osteogenic cells under adipogenic conditions

Mouse Models of Human Disease
OMIM IDRef(s)
Osteopathia Striata with Cranial Sclerosis; OSCS 300373 J:173242