Article
Mutant cartilage oligomeric matrix protein (COMP) compromises bone integrity, joint function and the balance between adipogenesis and osteogenesis.
Matrix biology : journal of the International Society for Matrix Biology - 1 Apr 2018
Coustry Francoise, Posey Karen L, Maerz Tristan, Baker Kevin, Abraham Annie M, Ambrose Catherine G, Nobakhti Sabah, Shefelbine Sandra J, Bi Xiaohong, Newton Michael, Gawronski Karissa, Remer Lindsay, Veerisetty Alka C, Hossain Mohammad G, Chiu Frankie, Hecht Jacqueline T
Abstract excerpt
Mutations in COMP (cartilage oligomeric matrix protein) cause severe long bone shortening in mice and humans. Previously, we showed that massive accumulation of misfolded COMP in the ER of growth plate chondrocytes in our MT-COMP mouse model of pseudoachondroplasia (PSACH) causes premature chondrocyte death and loss of linear growth. Premature chondrocyte death results from activation of oxidative stress and...
Topics
- Achondroplasia
- Adipogenesis
- Animals
- Bone Density
- Cartilage Oligomeric Matrix Protein
- Disease Models, Animal
- Humans
- Mice
- MicroRNAs