The nature of fibrous dysplasia View Full Text


Ontology type: schema:ScholarlyArticle      Open Access: True


Article Info

DATE

2009-12

AUTHORS

Liviu Feller, Neil H Wood, Razia AG Khammissa, Johan Lemmer, Erich J Raubenheimer

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. More... »

PAGES

22

Identifiers

URI

http://scigraph.springernature.com/pub.10.1186/1746-160x-5-22

DOI

http://dx.doi.org/10.1186/1746-160x-5-22

DIMENSIONS

https://app.dimensions.ai/details/publication/pub.1016666659

PUBMED

https://www.ncbi.nlm.nih.gov/pubmed/19895712


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