We found a match
Your institution may have rights to this item. Sign in to continue.
- Title
A murine model of neurofibromatosis type 1 tibial pseudarthrosis featuring proliferative fibrous tissue and osteoclast-like cells.
- Authors
El-Hoss, Jad; Sullivan, Kate; Cheng, Tegan; Yu, Nicole YC; Bobyn, Justin D; Peacock, Lauren; Mikulec, Kathy; Baldock, Paul; Alexander, Ian E; Schindeler, Aaron; Little, David G
- Abstract
Neurofibromatosis type 1 (NF1) is a common genetic condition caused by mutations in the NF1 gene. Patients often suffer from tissue-specific lesions associated with local double-inactivation of NF1. In this study, we generated a novel fracture model to investigate the mechanism underlying congenital pseudarthrosis of the tibia (CPT) associated with NF1. We used a Cre-expressing adenovirus (AdCre) to inactivate Nf1 in vitro in cultured osteoprogenitors and osteoblasts, and in vivo in the fracture callus of Nf1 flox/flox and Nf1 flox/− mice. The effects of the presence of Nf1 null cells were extensively examined. Cultured Nf1 null-committed osteoprogenitors from neonatal calvaria failed to differentiate and express mature osteoblastic markers, even with recombinant bone morphogenetic protein-2 (rhBMP-2) treatment. Similarly, Nf1 null-inducible osteoprogenitors obtained from Nf1
- Subjects
NEUROFIBROMATOSIS; PSEUDARTHROSIS; TIBIA; ADENOVIRUSES; LABORATORY mice
- Publication
Journal of Bone & Mineral Research, 2012, Vol 27, Issue 1, p68
- ISSN
0884-0431
- Publication type
Article
- DOI
10.1002/jbmr.528