The nature of fibrous dysplasia

被引：62

作者：

Feller L. ^{[1
]}

Wood N.H. ^{[1
]}

Khammissa R.A. ^{[1
]}

Lemmer J. ^{[1
]}

Raubenheimer E.J. ^{[2
]}

机构：

[1] Department of Periodontology and Oral Medicine, School of Dentistry, University of Limpopo (Medunsa Campus), Pretoria

[2] Department of Oral Pathology, School of Dentistry, University of Limpopo (Medunsa Campus), Pretoria

来源：

Head & Face Medicine | / 5卷 / 1期

关键词：

Acromegaly; Fibrous Dysplasia; Osteogenic Cell; Bony Lesion; Craniofacial Bone;

D O I：

10.1186/1746-160X-5-22

中图分类号：

学科分类号：

摘要：

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. © 2009 Feller et al.

引用

共 20 条

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