The nature of fibrous dysplasia. - 7thSpace Interactive

The nature of fibrous dysplasia.

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 Gs-alpha 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.

Author: Liviu FellerNeil WoodRazia KhammissaJohan LemmerErich Raubenheimer
Credits/Source: Head &Face Medicine 2009, 5:22

Published on: 2009-11-09

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