Article
Toward an understanding of the short bone phenotype associated with multiple osteochondromas.
Journal of orthopaedic research : official publication of the Orthopaedic Research Society - 1 Apr 2013
Jones Kevin B, Datar Manasi, Ravichandran Sandhya, Jin Huifeng, Jurrus Elizabeth, Whitaker Ross, Capecchi Mario R
Abstract excerpt
Individuals with multiple osteochondromas (MO) demonstrate shortened long bones. Ext1 or Ext2 haploinsufficiency cannot recapitulate the phenotype in mice. Loss of heterozygosity for Ext1 may induce shortening by steal of longitudinal growth into osteochondromas or by a general derangement of physeal signaling. We induced osteochondromagenesis at different time points during skeletal growth in a mouse genetic...
Topics
- Animals
- Disease Models, Animal
- Doxycycline
- Exostoses, Multiple Hereditary
- Femur
- Growth Plate
- Haploinsufficiency
- Loss of Heterozygosity
- Male
- Mice
- N-Acetylglucosaminyltransferases
- Phenotype
- Tibia
- Exostosin 1