The nature of fibrous dysplasia
Open Access
- 9 November 2009
- journal article
- review article
- Published by Springer Science and Business Media LLC in Head & Face Medicine
- Vol. 5 (1), 22
- https://doi.org/10.1186/1746-160x-5-22
Abstract
Fibrous dysplasia has been regarded as a developmental skeletal disorder characterized by replacement of normal bone with benign cellular fibrous connective tissue. It has now become evident that fibrous dysplasia is a genetic disease caused by somatic activating mutation of the Gsα subunit of G protein-coupled receptor resulting in upregulation of cAMP. This leads to defects in differentiation of osteoblasts with subsequent production of abnormal bone in an abundant fibrous stroma. In addition there is an increased production of IL-6 by mutated stromal fibrous dysplastic cells that induce osteoclastic bone resorption.Keywords
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