title: The nature of fibrous dysplasia.
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 Gsalpha 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.
authors.affiliation: Department of Periodontology and Oral Medicine, University of Limpopo, Pretoria, South Africa. lfeller@ul.ac.za
authors.forename: Liviu
authors.initials: L
authors.lastname: Feller
authors.forename: Neil H
authors.initials: NH
authors.lastname: Wood
authors.forename: Razia A G
authors.initials: RA
authors.lastname: Khammissa
authors.forename: Johan
authors.initials: J
authors.lastname: Lemmer
authors.forename: Erich J
authors.initials: EJ
authors.lastname: Raubenheimer
mesh_terms: D002454:Cell Differentiation; D005357:Fibrous Dysplasia of Bone / Q000000981:diagnostic imaging / Q000209:etiology / Q000235:genetics / Q000473:pathology; D006801:Humans; D009154:Mutation; D010006:Osteoblasts / Q000473:pathology; D011859:Radiography