The nature of fibrous dysplasia

Liviu Feller*, Neil H. Wood, Razia Ag Khammissa, Johan Lemmer, Erich J. Raubenheimer

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review

56 Citations (Scopus)

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 Gs 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.

Original languageEnglish
Article number22
JournalHead and Face Medicine
Volume5
Issue number1
DOIs
Publication statusPublished - 2009

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