Article
A mouse model of osteochondromagenesis from clonal inactivation of Ext1 in chondrocytes.
Proceedings of the National Academy of Sciences of the United States of America - 2 Feb 2010
Jones Kevin B, Piombo Virginia, Searby Charles, Kurriger Gail, Yang Baoli, Grabellus Florian, Roughley Peter J, Morcuende Jose A, Buckwalter Joseph A, Capecchi Mario R, Vortkamp Andrea, Sheffield Val C
Abstract excerpt
We report a mouse model of multiple osteochondromas (MO), an autosomal dominant disease in humans, also known as multiple hereditary exostoses (MHE or HME) and characterized by the formation of cartilage-capped osseous growths projecting from the metaphyses of endochondral bones. The pathogenesis of these osteochondromas has remained unclear. Mice heterozygous for Ext1 or Ext2, modeling the human genotypes that...
Topics
- Animals
- Bone Neoplasms
- Cell Proliferation
- Chondrocytes
- Disease Models, Animal
- Exons
- Exostoses, Multiple Hereditary
- Gene Targeting
- Homozygote
- Humans
- Loss of Heterozygosity
- Mice
- Mice, Transgenic