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

involves: 129S4/SvJae * C57BL/6 * SJL
Key:
phenotype observed in females WTSI Wellcome Trust Sanger Institute
phenotype observed in males EuPh Europhenome
N normal phenotype
       
skeleton
• recapitulates appendicular skeletal phenotypes seen in germline null mice (J:173242)
• sharp reduction in the adipocyte content of long bones (J:173242)
• asymmetrical distribution of ossification centers (J:173242)
• 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 (J:173242)
• decrease in the late differentiation of osteoblasts in primary bone marrow derived mesenchymal progenitor cell cultures (J:173242)
• primary bone marrow derived mesenchymal progenitor cell cultures continue to produce osteogenic cells under adipogenic conditions (J:173242)
• accumulation of cortical bone in adults (J:173242)
• increase in the osteoblast number relative to surface area (J:173242)
• accumulation of trabecular bone in adults (J:173242)
(J:173242)
• increased bone formation rate per tissue volume (J:173242)
• increase in bone mineralized surface per bone surface (J:173242)
• increase in osteoid volume per bone volume indicates a delay in bone mineralization (J:173242)

adipose tissue
• sharp reduction in the adipocyte content of long bones (J:173242)
• severely compromised formation of oil red O-positive adipocytes in primary bone marrow derived mesenchymal progenitor cell cultures (J:173242)

limbs/digits/tail

cellular
• severely compromised formation of oil red O-positive adipocytes in primary bone marrow derived mesenchymal progenitor cell cultures (J:173242)
• 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 (J:173242)
• decrease in the late differentiation of osteoblasts in primary bone marrow derived mesenchymal progenitor cell cultures (J:173242)
• primary bone marrow derived mesenchymal progenitor cell cultures continue to produce osteogenic cells under adipogenic conditions (J:173242)

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