Fibrodysplasia ossificans progressiva: a clue from the fly?

Calcified Tissue International
F S KaplanM A Zasloff

Abstract

Fibrodysplasia (myositis) ossificans progressiva (FOP) is a rare autosomal dominant disorder characterized by symmetrical congenital malformations of the blastemal anlage of the hands and feet and by progressive heterotopic chondrogenesis and ossification of the soft connective tissues. There is neither an established pathogenesis nor an effective treatment for this disabling disorder. We reevaluated the published data on the natural history of FOP and discovered an array of developmental gradients (characteristic patterns of disease expression) similar to developmental anomalies induced by pleiotropic mutations of the decapentaplegic (dpp) locus in Drosophila melanogaster. The protein encoded by the dpp locus is a member of the transforming growth factor-beta (TGF-beta) family of molecules. It shares 75% sequence homology with the c-terminal region of two recently cloned human bone morphogenetic proteins (BMP-2A, BMP-2B), also members of the TGF-beta family. The striking sequence identity across so large an evolutionary distance suggests that the BMP-2 genes in man and the dpp gene in the fly may be derived from a common ancestral gene. BMP is the only molecule discovered thus far that is capable of inducing endochondral ossif...Continue Reading

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Citations

Dec 17, 1998·Journal of Biomedical Materials Research·J H Brekke, J M Toth
Mar 1, 1992·Calcified Tissue International·J T Campbell, F S Kaplan
Nov 4, 2008·Journal of Bone and Mineral Metabolism·Frederick S KaplanEileen M Shore
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Apr 1, 1996·Otolaryngology--head and Neck Surgery : Official Journal of American Academy of Otolaryngology-Head and Neck Surgery·H B JanoffF S Kaplan
Aug 22, 1996·The New England Journal of Medicine·A B ShafritzF S Kaplan
Nov 2, 2012·Disease Models & Mechanisms·Frederick S KaplanEileen M Shore
Nov 25, 2000·Journal of Bone and Mineral Research : the Official Journal of the American Society for Bone and Mineral Research·F S Kaplan, E M Shore
Dec 28, 2012·Diseases of Aquatic Organisms·Luciano F La SalaMarcela M Uhart
May 9, 2013·Case Reports in Genetics·Rafael Herrera-EsparzaEsperanza Avalos-Díaz
Aug 12, 2014·Reumatología clinica·Antonio Morales-PigaGonzalo Sánchez-Duffhues
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Jun 9, 2015·Bone·Gonzalo Sánchez-DuffhuesPeter Ten Dijke
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May 20, 2000·Clinical Orthopaedics and Related Research·E M ShoreF H Gannon

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